Mantle Cell Lymphoma: NCCN Guidelines For Patients

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Mantle Cell
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N C C N N O N-H O D G K I N’S LY M P H O M A S E R I E S
Presented with support from:
Available online at NCCN.org/patients

Ü
Mantle Cell Lymphoma
LEARNING that you have cancer

can be overwhelming.
The goal of this book is to help you get the best cancer treatment. It explains
which cancer tests and treatments are recommended by experts of mantle cell
lymphoma.
The National Comprehensive Cancer Network® (NCCN®) is a not-for-profit

alliance of 27 leading cancer centers. Experts from NCCN have written treatment
guidelines for doctors who treat mantle cell lymphoma. These treatment guidelines
suggest what the best practice is for cancer care. The information in this patient
book is based on the guidelines written for doctors.
This book focuses on the treatment of mantle cell lymphoma. Key points of the
book are summarized in the NCCN Quick Guide™. NCCN also offers patient
books on diffuse large B-cell lymphoma, chronic lymphocytic leukemia, chronic
myelogenous leukemia, acute lymphoblastic leukemia, and many other cancer
types. Visit NCCN.org/patients for the full library of patient books, summaries, and
other resources.
NCCN Guidelines for Patients®:

Mantle Cell Lymphoma, 2017 1
About
These patient guidelines for cancer care are produced by the National
Comprehensive Cancer Network® (NCCN®).
The mission of NCCN is to improve cancer care so people can live better lives. At
the core of NCCN are the NCCN Clinical Practice Guidelines in Oncology (NCCN
Guidelines®). NCCN Guidelines® contain information to help health care workers
plan the best cancer care. They list options for cancer care that are most likely to
have the best results. The NCCN Guidelines for Patients® present the information
from the NCCN Guidelines in an easy-to-learn format.
Panels of experts create the NCCN Guidelines. Most of the experts are from
NCCN Member Institutions. Their areas of expertise are diverse. Many panels
also include a patient advocate. Recommendations in the NCCN Guidelines are
based on clinical trials and the experience of the panelists. The NCCN Guidelines
are updated at least once a year. When funded, the patient books are updated to
reflect the most recent version of the NCCN Guidelines for doctors.
For more information about the NCCN Guidelines, visit NCCN.org/clinical.asp.
Dorothy A. Shead, MS Laura J. Hanisch, PsyD Rachael Clarke

Director, Patient and Medical Writer/Patient Guidelines Data and
Clinical Information Information Specialist Layout Coordinator
Operations
Alycia Corrigan
Medical Writer
NCCN Foundation was founded by NCCN to raise funds for patient education
based on the NCCN Guidelines. NCCN Foundation offers guidance to people
with cancer and their caregivers at every step of their cancer journey. This is done
by sharing key information from leading cancer experts. This information can be
found in a library of NCCN Guidelines for Patients® and other patient education
resources. NCCN Foundation is also committed to advancing cancer treatment
by funding the nation’s promising doctors at the center of cancer research,
education, and progress of cancer therapies.
For more information about NCCN Foundation, visit NCCNFoundation.org.
© 2017 National Comprehensive Cancer Network, Inc. Based on the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®)
B-cell Lymphomas (Version 5.2017). Posted 9/26/2017.
All rights reserved. NCCN Guidelines for Patients® and illustrations herein may not be reproduced in any form for any purpose without
the express written permission of NCCN. The NCCN Guidelines are a work in progress that may be redefined as often as new significant
data become available. NCCN makes no warranties of any kind whatsoever regarding its content, use, or application and disclaims any
responsibility for its application or use in any way.
National Comprehensive Cancer Network (NCCN) • 275 Commerce Drive, Suite 300 • Fort Washington, PA 19034 • 215.690.0300

Supporters
Endorsed by
The Leukemia & Lymphoma Society (LLS)

LLS is dedicated to developing better outcomes for blood cancer patients through research, education and patient services
and is happy to have this comprehensive resource available to patients. LLS.org/informationspecialists.

Mantle Cell Lymphoma
Contents
6 How to use this book
7 Part 1
Mantle cell lymphoma basics
Explains how and where this cancer starts.
15 Part 2
Treatment planning
Describes how doctors plan your treatment.
24 Part 3
Overview of cancer treatments
Describes the treatments used to cure or control mantle cell lymphoma.
36 Part 4
Treatment guide
Presents treatment options based on the cancer stage.
46 Part 5
Making treatment decisions
Offers tips for choosing the best treatment.
54 Glossary
Dictionary
Acronyms
62 NCCN Panel Members
63 NCCN Member Institutions
64 Index

How to use this book
Who should read this book? Does this book include all
options?
Mantle cell lymphoma is the focus of this book.
Information on diagnosis, treatment planning, and This book includes information for many people.
treatment options are included. People with mantle Your treatment team can point out what applies to
cell lymphoma and those who support them— you. They can also give you more information. While
caregivers, family, and friends—may find this book reading, make a list of questions to ask your doctors.
helpful. It is a good starting point to learn what your
options may be. The treatment options are based on science and
the experience of NCCN experts. However, their
recommendations may not be right for you. Your
doctors may suggest other options based on your
Are the book chapters in a health and other factors. If other options are given,
certain order? ask your treatment team questions.
Early chapters explain concepts that are repeated

in later chapters. Starting with Part 1 may help. It
explains what mantle cell lymphoma is. Knowing Help! What do the words
more about this lymphoma may help you better mean?
understand its treatment.
In this book, many medical words are included.
Parts 2 through 5 address issues related to These are words that your treatment team may say
treatment. Part 2 lists which health tests and other to you. Most of these words may be new to you. It
steps of care are needed before treatment. Part 3 may be a lot to learn.
briefly describes all the types of treatments so you
can understand your options that are listed in Part 4. Don’t be discouraged as you read. Keep reading and
Tips for talking and deciding your options with your review the information. Ask your treatment team to
doctor are presented in Part 5. explain a word or phrase that you do not understand.
Words that you may not know are defined in the text
or in the Dictionary. Acronyms are also defined when
first used and in the Glossary. Acronyms are short
words formed from the first letters of several words.
One example is DNA for deoxyribonucleic acid.

1
Mantle cell lymphoma basics
8 Lymphatic system
8 A disease of cells
10 Diagnosis
14 Review

1 Mantle cell lymphoma basics Lymphatic system | A disease of cells
You’ve learned that you have or may have Lymphoid tissues

mantle cell lymphoma. It’s common to feel As lymph travels, it will pass through and be filtered
shocked and confused. Part 1 reviews by lymph nodes. Lymph nodes are organized masses
some basics that may help you learn of lymphoid tissue. There are hundreds of lymph
about mantle cell lymphoma. nodes throughout your body. See Figure 1. High
numbers of lymph nodes exist in the middle of your
chest, neck, armpit, groin, pelvis, and along your gut.
Lymph nodes and other lymphoid tissue are defined

Lymphatic system by high numbers of lymphocytes. Lymph also has
lymphocytes. Lymphocytes are a type of white
Before learning about mantle cell lymphoma, it is blood cell. They help fight germs. The three types of
helpful to know about the lymphatic system. It is lymphocytes are NK (natural killer) cells, B-cells, and
one of 13 systems of the human body. It transports T-cells. Lymphocytes are made in bone marrow then
fluids to the bloodstream and fights germs. As such, are moved by blood to the lymphatic system.
it supports your blood-flowing (cardiovascular) and
disease-fighting (immune) systems. Other parts of your body that have many
lymphocytes are included in the lymphatic system.
Lymph In children, the thymus stores T-cells until they are
Cells are the building blocks of tissue in the body. able to fight germs. Germs in blood are filtered and
The spaces between cells are filled with fluid. This destroyed by lymphocytes within your spleen. Your
fluid is called interstitial or tissue fluid. Most tissue tonsils kill germs in lymph that enter through your
fluid comes from parts of blood plasma that have mouth and nose. There are also small clumps of
passed out of blood vessels. Cells also release lymphatic tissue in your gut, thyroid, breasts, lungs,
waste and other products into tissue fluid. eyes, and skin.
When tissue fluid increases, it drains into vessels.

Almost all of tissue fluid drains back into blood
vessels. The rest of it drains into lymph vessels. A disease of cells
Once inside of lymph vessels, tissue fluid is called
lymph. Lymph travels in lymph vessels back to the Your body is made of trillions of cells. Cancer is a
bloodstream. disease of cells. Each type of cancer is named after
the cell from which it derived.
The lymphatic system also collects fat and some
vitamins from your gut. After you eat, your stomach Lymphoma
turns food into a liquid. Then, the liquid drains into Lymphomas are cancers of lymphocytes within
your small intestine. Within your small intestine, fat the lymphatic system. There are two main types of
and some vitamins are absorbed into lymph vessels. lymphomas. Hodgkin lymphoma is defined by the
This fatty lymph, called chyle, travels in lymph presence of Reed-Sternberg or related cells. Non-
vessels to the bloodstream. Hodgkin’s lymphoma includes all the other types of
lymphoma.

1 Mantle cell lymphoma basics A disease of cells
Most non-Hodgkin’s lymphomas—85 out of every pollen. Some antigens are formed inside your body
100—are B-cell lymphomas. About 10 out of 100 are like those found on tissue cells. Antibodies attach
T-cell lymphomas. A few have unknown cell origin. to antigens, which triggers a response from your
It is now known that most Hodgkin lymphomas are immune system.
also from B-cells. Thus, Hodgkin and non-Hodgkin’s
lymphomas are more related than first thought. Naïve B-cells
Germinal centers are short-lived structures within
Mantle cell lymphoma lymph nodes. They form in response to an outside
Mantle cell lymphoma is a type of non-Hodgkin’s antigen. Within germinal centers, B-cells undergo
lymphoma. It is a cancer of B-cells. There are many changes to prepare them for making antibodies.
types of B-cells and, thus, many B-cell cancers.
B-cells differ from one another based on the cell’s Most mantle cell lymphomas start from B-cells that
stage of development. As B-cells “mature” they have not passed through a germinal center. These
change in their ability to make antibodies. “naïve” B-cells can be found in mantle zones that
surround germinal centers. Of interest, a subset of
Antibodies are Y-shaped proteins that are made mantle cell lymphomas appears to start from B-cells
in response to the presence of antigens. Some that have passed through germinal centers.
antigens enter your body from outside. Such
antigens include viruses, bacteria, chemicals, and
Figure 1
Lymphatic system
The lymphatic system kills

germs in the body and collects
and transports lymph to the
bloodstream.
Illustration Copyright © 2017 Nucleus Medical Media, All rights reserved. www.nucleusinc.com

1 Mantle cell lymphoma basics Diagnosis
Mutations Diagnosis
Cells have a control center called the nucleus.
The nucleus contains chromosomes, which are One of the first signs of mantle cell lymphoma may
long strands of DNA (deoxyribonucleic acid) tightly be a swelling of lymph nodes. These nodes may
wrapped around proteins. See Figure 2. Within be in your neck, armpit, or groin area. The cancer
DNA are coded instructions for building new cells and may also affect your GI (gastrointestinal) tract and
controlling how cells behave. These instructions are bone marrow. GI symptoms include pain in your gut,
called genes. diarrhea, and bloody stools. Tests needed to confirm
(diagnose) mantle cell lymphoma are described next.
There can be abnormal changes in genes called
mutations. Some types of mutations that are linked Biopsy
to cancer are present in all cells. Other mutations are The only way to know if you have cancer is to test
present only in cancer cells. Mutations cause cancer tissue or fluid. A biopsy is a procedure that removes
cells to not behave like normal cells and, sometimes, samples of fluid or tissue for testing. There are many
to look very different from normal cells. Researchers types of biopsy.
are still trying to learn what causes genes to mutate
and cause cancer. For mantle cell lymphoma, an incisional or excisional
biopsy is advised. An incisional biopsy removes only
Cancer’s threat a part of the tumor through a cut made into your
Cancer cells don’t behave like normal cells. First, the body. An excisional biopsy removes the whole tumor
mutations cause cancer cells to grow more quickly and not much else. The methods used to do either
and live longer than normal cells. Normal cells grow biopsy depend on where the tumor is in your body.
and then divide to form new cells when needed.
They also die when old or damaged as shown in FNA (fine-needle aspiration) and a core needle
Figure 3. In contrast, cancer cells make new cells biopsy remove very small samples with a needle.
that aren’t needed and don’t die quickly when old or Neither should be used alone to diagnosis mantle
damaged. Over time, the lymphoma cells may build cell lymphoma. You may have cancer even if these
up in tissues and may travel in blood or lymph to biopsies find no cancer.
other sites. Without treatment, the cancer may cause
organs not to work. Hematopathology review
The biopsy samples will be sent to a special type
of pathologist. A pathologist is a doctor who’s an
expert in testing cells to find disease. For mantle cell
lymphoma, the pathologist should be a specialist in
hematopathology.
Hematopathologists spend all of their time looking

at blood, bone marrow, and lymph nodes. They
become very good at diagnosing blood cancers. The
hematopathologist will first examine the samples
using a microscope.

Figure 2
Genetic material in cells
Most human cells contain the

“blueprint of life”—the plan by
which our bodies are made and
work. The plan is found inside of
chromosomes, which are long
strands of DNA that are tightly
wrapped around proteins. Genes
are small pieces of DNA that
contain instructions for building
new cells and controlling how
cells behave. Humans have an
estimated 20,000 to 25,000 genes.
Figure 3
Normal cell growth vs.
cancer cell growth
Normal cells increase in number

when they are needed and
die when old or damaged. In
contrast, cancer cells quickly
make new cells and live longer
because of abnormal changes in
genes.

The hematopathologist will assess the rate of cancer IHC panel

growth by noting how the cells look. Compared to The IHC panel should test for BCL2, BCL6, CD3,
other lymphomas, most mantle cell lymphomas don’t CD5, CD10, CD20, CD21, CD23, cyclin D1, and
grow slowly but don’t grow very fast either. Doctors Ki-67. Mantle cell lymphoma cells often have CD5,
describe the growth as “moderately aggressive.” CD20, CD43, and high levels of cyclin D1. Rare
However, there are some mantle cell lymphomas that cases may have normal cyclin D1 levels. CD10
grow slowly and others that grow quite fast. and CD23 are found on most but not all mantle cell
lymphoma cells.
Protein tests
For diagnosis, the hematopathologist needs to study Sometimes it is helpful to include LEF1 and SOX11
the proteins in the cells’ surface (membrane). This is in the IHC panel. If LEF1 is found, the cancer is likely
called immunophenotyping. Mantle cell lymphoma chronic lymphocytic leukemia and not mantle cell
has a common pattern or “signature” of proteins. lymphoma. SOX11 is found in almost every case
See Figure 4. An IHC (immunohistochemistry) of mantle cell lymphoma. Thus, SOX11 is helpful
panel is a test for such proteins. It involves applying for diagnosis when the cells have normal levels of
a chemical marker to cells and then looking at them cyclin D1. On the other hand, a low or absent SOX11
with a microscope. level is a good marker of slow-growing mantle cell
lymphoma.
Figure 4
CD20 protein
Mantle cell lymphoma cells have

a common pattern of proteins
in their membrane. This pattern
includes the presence of CD5,
CD20, CD43, and high levels
of cyclin D1. CD10 and CD23
are found on most but not all
cells. Immunophenotyping is
the process of identifying the
proteins in cells’ membranes.
Derivative work of Rituximab Binding to CD20 on a B Cell Surface by NIAID available at commons.
wikimedia.org/wiki/File:Rituxima_Binding_to_CD20_on_a_B_Cell_Surface_(6830897205).jpg under a
Creative Commons Attribution 2.0 Generic license

Flow cytometry Genetic tests

Flow cytometry is a newer method that can also be Mantle cell lymphoma has common abnormal
used to assess the surface proteins on lymphoma changes in chromosomes and genes. A translocation
cells. This method involves first adding a marker—a is a switching of parts between two chromosomes.
light-sensitive dye—to cells. Then, your blood will A gene rearrangement is the fusion of one gene with
be passed through a flow cytometry machine. The another gene to create a new gene. Genetic testing
machine measures surface proteins on thousands of may be useful.
cells.
t(11;14)
Flow cytometry may be done in addition to an IHC Besides a protein signature, mantle cell lymphomas
panel. If done, it should test for CD5, CD10, CD19, often have a translocation. The translocation in
CD20, CD23, and kappa and lambda light chain mantle cell lymphoma occurs between chromosomes
proteins. Light chain proteins are part of antibodies. 11 and 14—referred to as t(11;14). See Figure 5.
It may be helpful to test for CD200 if the type of t(11;14) often involves the switching of the CCND1
cancer is unclear. Mantle cell lymphoma cells do not gene on chromosome 11 with the IgH locus on
have CD200. Chronic lymphocytic leukemia cells do. chromosome 14. The result is too much cyclin D1.
If cyclin D1 is normal on the IHC panel, it may be
useful to test for t(11;14).
Figure 5
t(11;14)
A translocation is a switching of
parts between chromosomes.
In mantle cell lymphoma
cells, a translocation between
chromosomes 11 and 14 is often
present. The result is too much
cyclin D1 on the surface of
cancer cells.
Copyright © 2017 National Comprehensive Cancer Network® (NCCN®). www.nccn.org

1 Mantle cell lymphoma basics Review
Tests that detect translocations are a karyotype or Review

FISH (fluorescence in situ hybridization).
The lymphatic system consists of lymph and
IGHV mutations or SOX11 a network of vessels and organs. It helps kill
Some mantle cell lymphomas have mutations in the germs in the body and transports fluids to the
IGHV (immunoglobulin heavy-chain variable) region bloodstream.
genes. These lymphomas appear to have started
from B-cells that have passed through germinal Lymphomas are cancers that start in
centers. They often have low or absent SOX11 lymphocytes within the lymphatic system.
levels. Mantle cell lymphoma is a cancer of B-cells. It
starts in B-cells that are from germinal centers
It may be useful to test for IGHV mutations since they found within lymphatic organs.
are a sign of slow-growing mantle cell lymphoma.
DNA sequencing is a lab test of blood or marrow Incisional or excisional biopsy is needed to
that is used to look for mutations in genes. This test diagnose mantle cell lymphoma.
reveals the order of the chemicals that make up
DNA. Most mantle cell lymphomas grow and spread
moderately fast. However, some do grow slowly
Other genetic errors and others very fast.
Other genetic tests may be useful if the type of
cancer is unclear. A karyotype or FISH to test for The biopsy tissue should be tested by a
translocation of chromosomes 14 and 18 may be hematopathologist. The hematopathologist will
done. This translocation is often found in follicular perform a number of tests that assess for cell
lymphoma and sometimes diffuse large B-cell type, surface proteins, and maybe genetics.
lymphoma. If present, the cancer is unlikely mantle
cell lymphoma. Likewise, the cancer is likely to be
chronic lymphocytic leukemia if a FISH panel shows
defects in chromosomes 11, 12, 13, or 17.
Pathology report
All lab results are recorded in a pathology report.
A report will be written each time tissue is removed
from your body and tested. These reports are vital to
diagnosis and planning treatment.
Review your pathology report(s) with your doctor. Ask

questions if you don’t understand. This information
can be complex. It’s also a good idea to get a copy of
your pathology report(s) and take notes.

2
Treatment planning
16 Medical history 23 Fertility and pregnancy
17 Physical exam 23 Review
17 Blood tests
18 Imaging tests
20 Scopes
21 Bone marrow exam
22 Spinal fluid tests
22 Heart tests

2 Treatment planning Medical history
Doctors plan treatment with many sources Symptoms are a part of your medical history. Some
of information. One of these sources is symptoms of mantle cell lymphoma are tiredness, a
tests of your health and the cancer. Part 2 feeling of fullness in your belly, and getting sick. This
describes who should receive which tests cancer may also cause “B symptoms.” It’s important
before treatment. Some of these tests that your doctor knows if you have them. These
are repeated during and after treatment. symptoms include fevers, chills, night sweats, and
Besides tests, Part 2 describes other weight loss without dieting.
types of care that are important to receive
before cancer treatment. Some cancers and other health conditions can run in
families. Thus, your doctor will ask about the medical
history of your blood relatives. You doctor may
ask about the health of your siblings, your parents
and their siblings, and your grandparents and their
Medical history siblings. Be prepared to tell who in your family has
had what diseases and at what ages.
Your medical history includes any health events and
medicines you’ve taken in your life. You will be asked A medical history is one of the tests needed for
about illnesses, injuries, health conditions, and more. treatment planning. See Guide 1 for a complete
It may help to make a list of old and new medications list of care that is recommended prior to treatment.
while at home to bring to your doctor’s office. Some types of care are for anyone with mantle cell
lymphoma while others may be useful for some
people.
Guide 1. Health care before cancer treatment
Must haves Sometimes useful
Medical history Beta-2 microglobulin

Physical exam Uric acid
CBC with differential CT scan of your neck
Comprehensive metabolic panel Endosccopy or colonoscopy
Lactate dehydrogenase Lumbar puncture in rare cases
Hepatitis B testing Fertility support
CT, whole-body PET/CT, or both
Bone marrow biopsy ± aspiration
Echocardiogram or MUGA if you will receive certain

types of chemotherapy
Pregnancy test if you can have babies

2 Treatment planning Physical exam | Blood tests
Physical exam Blood tests

Doctors should perform a physical exam along with Blood tests are used to learn if cancer treatment
taking a medical history. A physical exam is a study might be needed now. They are also used to
of your body for signs of disease. To start, your basic find unknown diseases including those related to
body functions will be measured. These functions lymphoma. It’s important to treat all illnesses.
include your temperature, blood pressure, and pulse
and breathing (respiration) rate. Your weight will also Blood tests require a sample of your blood. Samples
be checked. of blood can be removed with a blood draw. Before
a blood draw, you might need to stop drinking and
During the exam, your doctor will listen to your lungs, eating for several hours.
heart, and gut. Your doctor will also look at and feel
parts of your body. This is done to see if organs are A needle will be inserted into your vein to remove
of normal size, are soft or hard, or cause pain when blood. The needle may bruise your skin. You may
touched. Cancer and other health conditions can feel dizzy from the blood draw. Your blood sample
cause organs to become enlarged and hard. will be sent to a lab for testing.
Enlarged structures Complete blood count with differential

For mantle cell lymphoma, there are certain parts A CBC (complete blood count) measures parts of the
of your body that should be checked. Mantle cell blood. It is often done with a machine. Test results
lymphoma is often found in lymph nodes. Thus, include counts of white blood cells, red blood cells,
areas with lots of lymph nodes should be examined. and platelets. Your blood counts may be low or high
High numbers of lymph nodes exist in the middle of because of cancer or another health problem. It is
your chest, neck, throat, armpit, groin, pelvis, and an essential test that gives a picture of your overall
along your gut. The size of your spleen and liver health.
should also be assessed.
There are several types of white blood cells. A
Performance status differential counts the number of each type of cell.
Results of your medical history and physical exam It also checks if the counts are in balance with each
will be used to rate your performance status. other. Your doctor can determine the cause of an
Performance status is your ability to do daily abnormal white blood count from this test.
activities. It is used by doctors to assess if you can
undergo certain treatments. Comprehensive metabolic panel
Chemicals in your blood come from your liver, bone,
and other organs. A comprehensive metabolic panel
often includes tests for up to 14 chemicals. The tests
show if the levels of chemicals are too low or high.
Abnormal levels can be caused by cancer or other
health problems.
LDH
LDH (lactate dehydrogenase) is a protein that is
in most cells. It gets into your blood when a cell is

2 Treatment planning Imaging tests
damaged. Thus, a high level of LDH is a sign of Imaging tests

cell damage. High levels can be caused by cancer
or other health problems. If related to cancer, high Imaging tests make pictures (images) of the insides
levels may be a sign that treatment may be needed of your body. They can show which sites have
now or soon. cancer. This information helps your doctors stage
the cancer. More information on cancer staging is in
Beta-2 microglobulin Part 4.
Beta-2 microglobulin is a small protein made by
many types of cells, including lymphoma cells. It is Your treatment team will tell you how to prepare
measured with a blood chemistry test. If related to for the test. You may need to stop taking some
cancer, high levels may be a sign that treatment may medicines and stop eating and drinking for a few
be needed now or soon. hours before the scan. Tell your doctors if you get
nervous when in small spaces. You may be given a
Hepatitis testing sedative to help you relax.
Hepatitis B can be an important factor in the
treatment of mantle cell lymphoma. Hepatitis B can Imaging machines are large. You will likely be lying
become active again due to the cancer or some of its down during testing. At least part of your body will
treatments. Thus, tell your treatment team if you’ve be in the machine. Figure 6 shows a CT machine,
ever been infected with hepatitis. If you’re unsure, which is described next.
ask your treatment team if you should get tested.
Testing may be needed if you will be treated with a After the test, you will likely be able to resume your
drug called rituximab. activities right away. If you took a sedative, you will
have a waiting period. You may not learn of the
Uric acid results for a few days since a radiologist needs to
Some people with mantle cell lymphoma are at risk see the pictures. A radiologist is a doctor who’s an
for TLS (tumor lysis syndrome). This syndrome can expert in reading the images.
be life threatening. It occurs when the waste released
by dead cells is not quickly cleared out of your body. Diagnostic CT
This results in kidney damage and severe blood A CT (computed tomography) of your chest, belly
electrolyte disturbances. area, and between your hip bones is needed. A CT
of your neck is sometimes useful to learn if cancer is
TLS can occur among people with mantle cell present.
lymphoma who are undergoing strong cancer
treatments. The cancer treatment kills many cancer CT takes many pictures of a body part from different
cells. In turn, too much tumor cell waste is released angles using x-rays. A computer combines the x-rays
within in a short period of time. to make detailed pictures. The picture is saved for
later viewing by the radiologist.
Your doctors may want to know your uric acid level
before starting treatment. You may be given certain A contrast dye is used for diagnostic CT. It makes the
medications that can help prevent TLS. Also, drinking pictures clearer. The dye will be injected into a vein
plenty of water throughout chemotherapy can help. in your hand or arm. You will also be given a liquid
Ask your treatment team for more information. contrast to drink.

2 Treatment planning Imaging tests
The contrast may cause you to feel flushed or get

hives. Rarely, serious allergic reactions occur. Tell
your doctor and the technicians if you have had
Figure 6
problems with contrast in the past.
CT machine
Pictures of the insides of your body can

Whole-body PET/CT
be made with an imaging test. During the
Sometimes CT is combined with PET (positron
scan, you will lie on a table that will move
emission tomography). When used together, they
into the tunnel of the imaging machine.
are called a PET/CT scan. PET/CT may be done
The pictures will be viewed by a doctor
with one or two machines depending on the cancer
who will look for signs of cancer.
center.
For PET, a sugar radiotracer will be injected into

your body. The radiotracer is detected with a special
camera. Cancer cells appear brighter than normal
cells because they use sugar more quickly. PET/CT
may be done with one or two machines depending
on the cancer center.
Whole-body PET/CT may be given in addition to or

instead of CT. It can show the presence of cancer
when other tests do not. PET/CT is also used to
assess if treatment is working.
Copyright © 2017 National Comprehensive Cancer Network® (NCCN®).

www.nccn.org

2 Treatment planning Scopes
Scopes colonoscope. The picture from the colonoscope will

be viewed by your doctor on a screen.
It is common for mantle cell lymphoma to spread
to the GI tract. Endoscopy and colonoscopy are To prepare for this test, your doctor may place you on
procedures during which a “scope” is inserted into a liquid diet for 1 to 3 days. You may also be given a
the GI tract. The scope is used to remove tissue that laxative or an enema to clean out your intestine the
will be tested for cancer. night before the test. Right before the test, you may
be given a sedative. The day after the test, you are
Testing for cancer in your GI tract may not be likely to feel normal. If you have severe pain, bloody
needed. It may not be needed if your treatment stools, or weakness, contact your doctor.
options wouldn’t be affected. NCCN experts do
advise GI testing to confirm there’s only local
disease. A second scope will be done to assess how
well treatment worked for local disease.
Endoscopy
An endoscopy allows your doctor to see the insides
of your esophagus and stomach. It is done with an Figure 7
endoscope. An endoscope has a thin, long tube Colonoscopy
that will be guided into your body, often through the
mouth. At the end of the tube is a very small light, Tissue samples from your GI tract may
camera lens, and cutting tool. At the other end of be removed to test for cancer. The
the endoscope is an eyepiece. Your doctor will look tissue samples from your colon may
through it to see the images shown by the camera. be removed during a procedure called
a colonoscopy as shown below. An
You will likely be sedated during the exam, but endoscopy is a procedure that removes
sometimes general anesthesia is used. Air may be tissue samples from the upper GI tract,
pumped into your esophagus and stomach for better which includes your esophagus and
viewing. After the exam, your throat may feel sore stomach.
and you may feel bloated from the air.
Colonoscopy
A colonoscopy allows your doctor to see inside your
colon. It is done with a colonoscope, which looks and
works much like an endoscope. You will be asked to
wear a hospital gown and lie on your side during the
exam as shown in Figure 7.
The colonoscope will be inserted into your anus

and gently guided through your intestine. Air may
be pumped into your intestine to make it bigger Illustration Copyright © 2017 Nucleus Medical Media, All rights
for better viewing. You may be asked to shift a reserved. www.nucleusinc.com
little during the test to help your doctor guide the

2 Treatment planning Bone marrow exam
Bone marrow exam For aspiration, a hollow needle will be inserted into
your skin and pushed into the bone. Liquid bone
Mantle cell lymphoma is often but not always found marrow will then be drawn into a syringe. For the
in bone marrow at diagnosis. Thus, a bone marrow biopsy, a wider needle will be inserted into your bone
exam is advised. This exam will confirm if there is and rotated to remove a core sample.
cancer or not.
The samples will be sent to a lab for testing. You may
A bone marrow exam consists of two procedures. A feel bone pain during and after the procedures for a
bone marrow aspiration removes a small amount of few days. Your skin may bruise.
liquid bone marrow. A bone marrow biopsy removes
a sample of bone and soft bone marrow.
Often, these procedures are done at the same time.

They are performed on the back of hip bone. You
may receive a light sedative beforehand. You will
likely lie on your side as shown in Figure 8. Some
people lie on their belly. Your doctor will first clean
and numb your skin. Figure 8
“
Bone marrow exam
A bone marrow exam removes a sample

of bone and marrow for testing. The
procedure is often done on the back of
the hip.
My initial symptom was an

enlarged spleen. A bone marrow
biopsy determined I had mantle
cell lymphoma It is a good thing
my primary care doctor found the
enlarged spleen because I did not
have any of the usual symptoms.
– Scott
Survivor, Diagnosed at age 54
Illustration Copyright © 2017 Nucleus Medical Media, All rights

reserved. www.nucleusinc.com

2 Treatment planning Spinal fluid tests | Heart tests
Spinal fluid tests Echocardiogram

An echocardiogram is an imaging test of your heart.
Mantle cell lymphoma rarely spreads into the fluid It uses sound waves (ultrasound) to make pictures.
around the brain and spinal cord. This fluid is called During this test, you will be lying down. Small
cerebrospinal fluid or spinal fluid. It may spread patches will be placed on your chest to track your
for blastic variant subtype. It may have spread into heartbeat. Next, a probe with gel on its tip will be
your spinal fluid if certain symptoms are present. To slid across part of your bare chest. A picture of your
confirm that cancer is in spinal fluid, a sample must beating heart will be seen at once on a screen. The
be removed and tested. pictures will be recorded for future viewing.
A lumbar puncture is a procedure that removes MUGA

spinal fluid. It is also called a spinal tap. A lumbar A MUGA (multi-gated acquisition) scan measures
puncture is advised if you have the blastoid type of how well your heart is pumping blood. For this test,
mantle cell lymphoma or have symptoms of cancer in patches will be placed on your chest to track your
your CNS (central nervous system). Such symptoms heartbeat. Also, a radiotracer will be injected into
include a loss of balance, loss of sensation, muscle
weakness, seizures, headaches, and vision changes.
During a spinal tap, you will be lying down or sitting

Figure 9
on an exam table. If lying down, your knees must
Lumbar puncture
be tucked up near your chest. If sitting, you must
lean slightly forward and down toward your knees as
A lumbar puncture is used to remove a
shown in Figure 9.
sample of spinal fluid so it can be tested
for cancer. A lumbar puncture may also
The lower part of your back over your spine will be
be used to inject cancer drugs into spinal
numbed with a local anesthetic. Next, a thin needle
fluid.
will be inserted between the bones of your spine and
into the space around your spinal cord. You may
feel some pressure during the procedure. The fluid
sample will then be sent to a lab for testing.
Heart tests
Some cancer treatments can damage your heart.
Thus, your doctor may test how well your heart works
to plan treatment. You may receive one of the two
tests described next. If your heart isn’t working well,
you may receive other treatment.
Illustration Copyright © 2017 Nucleus Medical Media, All rights
reserved. www.nucleusinc.com

2 Treatment planning Fertility and pregnancy | Review
your vein. Pictures of your heart will be taken with a Review

special camera that can detect the radiation released
by the tracer. Tell your doctor if you have recently had
fevers, night sweats, and weight loss without
dieting. These can be symptoms of mantle cell
lymphoma.
Fertility and pregnancy
Your doctor will examine your body for signs
Some cancer treatments can limit your ability to have of disease. He or she will check if your lymph
a baby. If you want the choice of having babies after nodes, liver, or spleen are large. Your doctor will
treatment or are unsure, tell your doctors. It may also also rate your ability to do everyday activities.
help to talk with a fertility specialist before you begin
cancer treatment. Blood tests can be done to assess if cancer
treatment is needed and for other health
A fertility specialist is an expert in helping people conditions.
have babies. The fertility specialist can discuss
with you how to have a baby after treatment. Some Imaging tests allow your doctors to see inside
methods of fertility preservation are discussed next. your body without cutting into it. CT, PET/CT, or
If you are a woman of childbearing age, important both is needed.
information on pregnancy is also addressed.
Endoscopy or colonoscopy may be done to
Sperm banking collect tissue samples from your GI tract in
Men who want to father children after cancer order to confirm local disease and treatment
treatment can use sperm banking. Sperm banking response.
stores semen for later use. This is done by freezing
semen with sperm in liquid nitrogen. Talk to your A bone marrow biopsy removes a piece of bone
treatment team about the costs of and how well and marrow to test for cancer cells. An aspirate
sperm banking works. removes liquid marrow. These tests may be
helpful before starting treatment.
Egg freezing and more
Like sperm banking, a woman’s eggs can be A lumbar puncture may be needed to confirm if
removed, frozen, and stored for later use. Your the cancer has spread into your spinal fluid.
frozen eggs can be fertilized with sperm beforehand.
Also, a part of your ovary that contains eggs can be You may undergo heart tests to see if you
frozen and stored. are healthy enough to have certain cancer
treatments.
Pregnancy test
Some cancer treatments can harm an unborn baby. Talk to a fertility specialist to learn about ways
Get a pregnancy test before treatment if you may be to have babies after cancer treatment. If you
pregnant now. Your treatment options will depend may be pregnant now, get a pregnancy test
on the results. During treatment, take steps to avoid since some cancer treatments can harm unborn
getting pregnant. Your doctors can tell you which babies.
birth control methods are best to use.

1
3
Chapter title
Overview of cancer
initial cap
treatments
only
25 Immunotherapy
x Header here initial cap only Guide 1
Guide 1 Guide 2
26 Immunomodulators
Guide 2 Guide 3
Guide 3
27 Chemotherapy
x Header here initial cap only
29 Steroids
x
30 Header
Targetedhere initial cap only
therapy
x
31 Header here
Radiation initial cap only
therapy
x
33 Header herecell
Blood stem initial cap only
transplant
x
34 Header
Clinical here
trialsinitial cap only
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35 Review

3 Overview of cancer treatments Immunotherapy
In Part 3, the main treatment types Immunotherapy is a common treatment for mantle
for mantle cell lymphoma are briefly cell lymphoma.
described. Knowing what a treatment is
will help you understand your treatment Immunotherapy for mantle cell lymphoma uses
options listed in Part 4. There is more than rituximab. It is an anti-CD20 monoclonal antibody.
one treatment for mantle cell lymphoma. This human-made antibody attaches to CD20 on the
Not every person will receive every surface of lymphoma cells. See Figure 10. It works
treatment described in this chapter. by marking the cells for destruction. It may directly kill
cells, too.
Rituximab is sold as Rituxan®. It is a liquid that will

be slowly injected into your vein. It often takes a few
Immunotherapy hours to receive the full dose. How often rituximab is
received differs among people. Ask your doctor for
The immune system is your body’s natural defense more information.
against disease. Immunotherapy increases the
activity of this system. As a result, it improves Rituximab is also sold as Rituxan Hycela™. This
your body’s ability to find and destroy cancer cells. medicine is injected under the skin for treatment
of certain lymphomas. Injections may be received
Figure 10
Anti-CD20 monoclonal
antibody
Anti-CD20 monoclonal antibodies

attach to lymphoma cells to mark
them for destruction by your
immune system.
Derivative work of Rituximab Binding to CD20 on a B Cell Surface by NIAID available at commons.
wikimedia.org/wiki/File:Rituxima_Binding_to_CD20_on_a_B_Cell_Surface_(6830897205).jpg under a
Creative Commons Attribution 2.0 Generic license

3 Overview of cancer treatments Immunomodulators
after the first dose of rituximab by infusion. Also, Immunomodulators

injections may be an option as long as rituximab isn’t
being used with ibritumomab tiuxetan. Injections last Immunomodulators are drugs that modify different
between 5 and 7 minutes. parts of the immune system. Lenalidomide
(Revlimid®) is an immunomodulator. It is received
Side effects are unhealthy or unpleasant physical with rituximab as a first-line treatment. For second-
or emotional responses to treatment. Ask your line treatment, rituximab or rituximab with ibrutinib
treatment team for a full list of common and rare side may be added.
effects.
Lenalidomide treats lymphoma in more than
You may have an allergic reaction while receiving one way. As an immunomodulator, it boosts the
rituximab. Other common side effects are chills, immune system. It also helps stop cancer cells from
infections, body aches, tiredness, and low blood cell increasing in number. Third, it also works like a type
counts. Rituximab also increases your chances for of targeted therapy called an angiogenesis inhibitor.
tumor lysis syndrome, heart problems, and blockage Angiogenesis inhibitors stop the growth of new blood
and tears in your gut. vessels that would provide food (nutrients) to the
cancer.
“
Lenalidomide is made in pill form. It is given in cycles
of treatment days followed by days of rest. A cycle
may consist of 3 weeks of treatment and 1 week of
rest. It may also be given for 4 straight weeks. Cycles
may repeat until the cancer grows or side effects
become severe.
Maintaining a positive attitude is
important. Reach out to your family Common side effects include low blood counts,
and friends for support. diarrhea, itching, rash, and fatigue. Serious but less
common side effects include blood clots, bleeding
– Scott disorders, loss of vision, and skin cancer. Ask your
Husband and survivor treatment team for a full list of side effects.

3 Overview of cancer treatments Chemotherapy
Chemotherapy Some chemotherapy drugs work when cells are in

an active growth phase. See Figure 11. During the
Chemotherapy, or “chemo,” includes drugs that active growth phase, cells grow and divide to form
disrupt the life cycle of cancer cells. The types of a new cell. Chemotherapy that disrupts the growth
chemotherapy differ in the way they work. Some kill phase works well for cancer cells that are growing
cancer cells by damaging their DNA or by disrupting and dividing quickly. Other chemotherapy drugs work
the making of DNA. Others interfere with cell parts in any growth or resting phase.
that are needed for making new cells. Thus, no new
cells are made to replace dying cells. Chemotherapy
can affect both cancer and normal cells.
Figure 11
Chemotherapy and the cell cycle
A cell goes through many changes to divide into two cells. Science has grouped these changes
into 7 main phases. There may be another phase of rest, too. Some chemotherapy drugs work in
any phase. Other chemotherapy drugs work in one or two growth phases. In growth phases, DNA
is copied and two full sets of chromosomes are made. A full set of chromosomes is pulled into
each end of the cell. The cell then divides into two cells each with their own set of chromosomes.
Chemotherapy may work in some or all phases of cell division.
Copyright © 2017 National Comprehensive Cancer Network® (NCCN®). www.nccn.org

3 Overview of cancer treatments Chemotherapy
What to expect Chemotherapy is given in cycles of treatment days

Chemotherapy used to treat mantle cell lymphoma followed by days of rest. This allows your body to
is listed in Guide 2. Most are liquids that are slowly recover before the next cycle. Cycles vary in length
injected into a vein. Some are a pill that is swallowed. depending on which drugs are used. Often, a cycle is
By any method, the drugs travel in your bloodstream 2 to 4 weeks long. If you will have chemotherapy, ask
to treat cancer throughout your body. Doctors use your doctor how many cycles will be given. Also ask
the term “systemic” when talking about a cancer how many days of treatment there are within a cycle.
treatment for the whole body.
Chemotherapy may consist of one or more drugs.
When only one drug is used, it is called a single
Guide 2. Chemotherapy
Generic (chemical) name Brand name (sold as)
Bendamustine hydrochloride Treanda®, Bendeka™

Bortezomib Velcade®
Carboplatin –
Carmustine BiCNU®
Cisplatin Platinol®
Cladribine Leustatin®
Cyclophosphamide –
Cytarabine Cytosar-U®
Doxorubicin hydrochloride –
Etoposide; Etoposide phosphate Etopophos® Preservative Free
Fludarabine Fludara®
Gemcitabine hydrochloride Gemzar®
Ifosfamide –
Methotrexate –
Mitoxantrone hydrochloride –
Oxaliplatin Eloxatin®
Pentostatin Nipent®
Procarbazine hydrochloride Matulane®
Vincristine sulfate –
Vinorelbine Navelbine®

3 Overview of cancer treatments Steroids
agent. However, not all drugs work the same way, Steroids
so often more than one drug is used. A combination
regimen is the use of two or more chemotherapy Steroid is the short name for corticosteroid. It
drugs. A steroid, immunotherapy, or both are often is a type of drug that is often used to relieve
added to chemotherapy. inflammation. Steroids also are toxic to lymphoma
cells. They have strong anti-cancer effects. The
Side effects of chemotherapy steroids used to treat mantle cell lymphoma are listed
Side effects are unhealthy or unpleasant physical or in Guide 3.
emotional responses to treatment. They differ among
people. Some people have many side effects. Other Steroids are a part of some chemotherapy
people have few. Some side effects can be very regimens. They are often given on the same days
serious. Others can be unpleasant but not serious. as chemotherapy but only for a few days or a week.
Prednisone is made in pill form but dexamethasone
Side effects of chemotherapy depend on multiple and methylprednisolone are made both as a liquid to
factors. These factors include the drug type, amount be injected or a pill to be swallowed.
taken, length of treatment, and the person. In
general, side effects are caused by the death of fast- Most side effects of steroids fade away once the
growing cells. drugs are stopped. Common side effects include
feeling hungry, upset stomach, and mood changes.
Fast-growing cells are found in the hair follicles, gut, You may have trouble sleeping. Wounds may be
mouth, and blood. Death of these cells can cause slow to heal. Swelling of ankles, feet, or hands is also
low blood cell counts, not feeling hungry, nausea, common.
vomiting, diarrhea, hair loss, and mouth sores. Lung
damage may also occur at the time of treatment.
Most side effects appear shortly after treatment Guide 3. Steroids

starts and will stop after treatment. However, other
side effects are long-term or may appear years later. Generic (chemical) Brand name
name (sold as)
Late side effects include another type of cancer,
heart disease, low levels of thyroid hormones Dexamethasone –
(hypothyroidism), and problems having babies
Methylprednisolone;
(infertility). A-Methapred
Methylprednisolone
Depo-Medrol®
acetate;
Not all side effects of chemotherapy are listed here. Medrol®
Methylprednisolone
Solu-Medrol®
Please ask your treatment team for a complete list of sodium succinate
common and rare side effects. If a side effect bothers
Prednisone –
you, tell your treatment team. There may be ways to
help you feel better. There are also ways to prevent
some side effects.

3 Overview of cancer treatments Targeted therapy
Targeted therapy Venetoclax

Venetoclax is sold as Venclexta™. It is a BCL-2
Targeted therapy is a class of drugs. These drugs inhibitor. BCL-2 is a protein inside of B-cells that
stop the action of molecules that help cancer cells helps prevent cell death.
grow. They are less likely to harm normal cells than
chemotherapy. Targeted therapy for mantle cell In some non-Hodgkin lymphomas, there is a large
lymphoma uses kinase or BCL-2 inhibitors. amount of BCL-2. BCL-2 inhibitors attach to BCL-2
and stop them from working. Thus, the lymphoma
Ibrutinib cells are able to die.
Ibrutinib is sold as Imbruvica®. It is a kinase inhibitor.
Kinases are molecules that move chemicals, called Venetoclax is made in pill form. It is taken once a
phosphates, from one molecule to another. Kinase day with food and water. Your doctor will tell you how
inhibitors stop the phosphate from being moved. many pills you need for your dose.
Ibrutinib stops a kinase called BTK (Bruton’s tyrosine Ask your treatment team for a full list of side effects
kinase). This kinase is found inside of B-cells. It of venetoclax. Some common side effects are
helps cells grow and survive. fatigue, diarrhea, nausea, and lung infection. Blood
counts are often low. Your doctor will assess for
Ibrutinib slows down mantle cell lymphoma. It works serious but uncommon side effects. Venetoclax may
by causing cell death. It also stops the cells from cause TLS.
quickly making new cells. Third, it stops the cells
from moving into your bone and marrow.
Ibrutinib is made in pill form. It is taken once a day Supportive care

around the same time. Your doctor will tell you how
many pills you need for your dose.
Supportive care doesn’t aim to treat cancer
Ask your treatment team for a full list of side effects
but aims to improve quality of life. It is
of ibrutinib. Some common side effects are fatigue,
diarrhea, muscle and bone pain, bruising, bleeding, also called palliative care. It can address
nausea, and rash. Blood counts are often low when many needs. One example is treatment
taking this medicine. However, there may be a short-
lived increase in white blood cells. for physical and emotional symptoms.
Supportive care can also help with treatment
Your doctor will assess for serious but uncommon
decisions as you may have more than one
side effects. These include heart and kidney
problems. You may develop serious bleeding and option. It can also help with coordination of
infections. Ibrutinib may increase your chances for care between health providers. Talk with your
other cancers.
treatment team to plan the best supportive
care for you.

3 Overview of cancer treatments Radiation therapy
Radiation therapy After simulation, your radiation team will further

plan your treatment. Plans are made by viewing
Radiation therapy uses high-energy x-rays to treat your scans on the treatment planning computer.
mantle cell lymphoma. The x-rays damage DNA in Your radiation oncologist will work closely with a
cancer cells. This either kills the cancer cells or stops dosimetrist. They will plan the best dose, number and
new cancer cells from being made. shape of radiation beams, and number of treatments.
Your plan will be designed to treat the cancer while
A radiation oncologist will oversee your radiation sparing normal tissue.
treatment. A radiation oncologist is a doctor who’s an
expert in treating cancer with radiation. He or she will Once your treatment plan is made, a setup session is
tailor treatment to you. needed. This session is sometimes called “port film”
day or dress rehearsal. The setup session occurs in
External beam radiation therapy the treatment room.
Mantle cell lymphoma may be treated with one of
two types of radiation therapy. ISRT (involved-site Treatment sessions
radiation therapy) can treat lymph nodes in which the During treatment, you will lie on a table in the same
cancer first started. It may also treat cancer near to position as done for simulation. Devices may be used
these nodes. Radiation delivered to a larger area is
called IFRT (involved-field radiation therapy).
Both are given with a method called EBRT (external Figure 12

beam radiation therapy). A large machine makes External beam radiation therapy
high energy x-rays used for treatment. This machine
is called a LINAC (linear accelerator). See Radiation therapy is often delivered from a
Figure 12. large machine called a linear accelerator. The
rays pass through skin and travel to the tumor.
Planning and setup sessions Healthy tissue is protected using modern types
A planning session is needed to map out your of treatment.
treatment. The planning process is called simulation.
It involves obtaining a scan of your body in the
position that is needed for treatment. The scan is
only used for treatment planning.
A CT scan with contrast is used. PET/CT and MRI

(magnetic resonance imaging) often enhance
treatment planning for ISRT. For tumors near the
breastbone, 4D-CT (four-dimensional computed
tomography) or fluoroscopy can account for tumor
movement from breathing. If your breathing causes
large movements, motion control methods during the
scans may be used.

3 Overview of cancer treatments Radiation therapy
to keep you from moving. You will be alone while fatigue. Changes in skin are also common right after
the therapists operate the machine from the nearby treatment. Your treated skin may look and feel as if
control room. it has a mild sunburn. It may also become dry, sore,
and feel painful when touched. You may also have
The therapists will be able to see, hear, and speak short-term hair loss, but only where treated.
with you. As treatment is given, you may hear noises.
One session takes less than 10 minutes. The types Treatment to the head and neck can cause mouth
of EBRT include: sores, dry mouth, changes in taste, and a sore
throat. Chest radiation can cause a dry cough or a
3D-CRT (three-dimensional conformal sensation of a lump when you swallow. Radiation
radiation therapy) delivers, from different near your belly can cause nausea and maybe
angles, a photon beam that matches the shape vomiting, and when given between your hip bones,
of the target. diarrhea and cramps.
IMRT (intensity-modulated radiation therapy) is Late effects

a form of 3D-CRT. It further modifies the beam’s Late effects are those that happen after treatment.
intensity during treatment. Some do not go away. The effects depend on
the treatment site. Examples include dry mouth,
Proton therapy uses proton beams that deliver dental cavities, hypothyroidism, lung scarring, heart
radiation mostly within the tumor. disease, infertility, and second cancers.
IGRT (image-guided radiation therapy) can improve Not all side effects of radiation are listed here.
how well the radiation beam targets some tumors. Please ask your treatment team for a complete list of
IGRT uses the machine that delivers the radiation common and rare side effects. If a side effect bothers
to also take images of the tumor and normal body you, tell your treatment team. There may be ways to
structures. This can be done right before or during help you feel better. There are also ways to prevent
treatment. These images are compared to the ones some side effects.
taken during simulation. If needed, changes will be
made to your body position or the radiation beams.
Side effects
Side effects from radiation therapy differ among
people. Factors like treatment site, radiation dose,
and length of treatment play a role. Side effects are
cumulative. This means they build up slowly and are
worse at the end of treatment. Your doctor will check
on you every week during treatment. He or she will
review skin care, medicines, and other options to
help you feel better.
Acute effects
Acute effects are those that happen during treatment
or shortly after the last session. Many people feel

3 Overview of cancer treatments Blood stem cell transplant
Blood stem cell transplant

Blood (hematopoietic) stem cells are cells from which Complementary and
all blood cells are formed. They mainly exist in bone alternative medicine
marrow. Cancer or its treatment can damage or
destroy blood stem cells.
CAM (complementary and alternative
A blood stem cell transplant replaces damaged or medicine) is a group of treatments that aren’t
destroyed stem cells with healthy stem cells. The
often given by doctors. There is much interest
healthy stem cells form new marrow and blood cells.
A transplant is sometimes an option for advanced today in CAM for cancer. Many CAMs are
mantle cell lymphoma. There are two types of blood being studied to see if they are truly helpful.
stem cell transplants.
Autologous blood stem cell transplant Complementary medicines are treatments

Autologous blood stem cell transplant uses your given along with usual medical treatments.
healthy stem cells to repair bone marrow. This
While CAMs aren’t known to kill cancer cells,
treatment is also called HDT/ASCR (high-dose
therapy with autologous stem cell rescue). Your they may improve your comfort and well-
healthy stem cells will be collected when imaging being. Two examples are acupuncture for
tests show that cancer treatment is working. You
will then receive intense chemotherapy and maybe
pain management and yoga for relaxation.
radiation to destroy any remaining cancer cells. This
intense treatment will also destroy bone marrow. Alternative medicine is used in place of usual
Your healthy stem cells will be put back into your
medicine. Some alternative medicines are
body to “rescue” your marrow.
sold as cures even though they haven’t been
Allogeneic blood stem cell transplant proven to work in clinical trials. If there was
Allogeneic blood stem cell transplant uses healthy
stem cells from a donor. HLA (human leukocyte good proof that CAMs or other treatments
antigen) typing is the test used to check if the donor cured cancer, they would be included in this
and your tissue type are a good fit. Chemotherapy
book.
will be given to destroy cancer cells and suppress
your immune system from attacking the donor cells.
It is important to tell your treatment team if
The transplanted stem cells will form new marrow you are using any CAMs. They can tell you
and attack remaining cancer cells. This attack is
known as the GVT (graft-versus-tumor) effect. On which CAMs may be helpful and which CAMs
the other hand, there is a serious risk of GVHD may limit how well medical treatments work.
(graft-versus-host disease). GVHD is when the
donated cells see the cells in your body as foreign
and attack them.

3 Overview of cancer treatments Clinical trials
Clinical trials Phase IV trials test drugs approved by the U.S.

FDA (Food and Drug Administration) to learn
One of your treatment choices may be to join more about side effects with long-term use.
a clinical trial. Joining a clinical trial is strongly
supported. NCCN believes that you will receive the Joining a clinical trial has benefits. First, you’ll have
best management in a clinical trial. access to the most current cancer care. However,
please note that it is unknown how well new
New tests and treatments aren’t offered to the treatments work if at all. Second, you will receive the
public as soon as they’re made. They first need to best management of care. Third, the results of your
be studied. A clinical trial is a type of research that treatment—both good and bad—will be carefully
studies a test or treatment in people. tracked. Fourth, you may help other people who will
have cancer in the future.
Clinical trials study how safe and helpful tests and
treatments are for people. When found to be safe Clinical trials have risks, too. Like any test or
and helpful, they may become tomorrow’s standard treatment, there may be side effects. Also, new
treatment. Because of clinical trials, the tests and tests or treatments may or may not improve your
treatments in this book are now widely used to help health. In fact, your health may worsen during a trial.
people with lymphoma. Future tests and treatments Other downsides may include more hospital trips,
that may have better results will depend on clinical paperwork, and extra costs for you.
trials.
To join a clinical trial, you must meet the conditions
New tests and treatments go through a series of of the study. Patients in a clinical trial are often alike
clinical trials. These trials aim to ensure they’re safe in terms of their cancer and general health. Thus, if
and work. Without clinical trials, there is no way to patients improve, it’s because of the treatment and
know if a test or treatment is safe or helpful. Clinical not because of differences between them.
trials have four phases. Some examples of the four
phases for treatment are: To join, you’ll need to review and sign an informed
consent form. This form describes the study in detail.
Phase I trials aim to find the safest and best The study’s risks and benefits should be described
dose of a new drug. Another aim is to find the and may include others than those described above.
best way to give the drug with the fewest side
effects. These trials often involve about 20 Ask your treatment team if there is an open clinical
people. trial that you can join. There may be clinical trials
where you’re getting treatment or at other treatment
Phase II trials assess if a drug works for centers nearby. You can also find clinical trials
a specific type of cancer. These trials often through the websites listed in Part 5.
involve 20 to 100 people.
Phase III trials compare a new drug to a

standard treatment head-to-head. These trials
often involve hundreds or thousands of people.

“
3 Overview of cancer treatments Review
Review
Immunotherapy improves your body’s ability to
find and destroy cancer cells.
My first chemo was rather harsh.
Immunomodulators treat lymphoma by The second chemo was was so
modifying your immune system and by other
much easier. That is the progress of
means.
newer, better chemo drugs.
Chemotherapy stops the life cycle of cancer – Scott
cells so they can’t increase in number.
Survivor, Relapsed at age 60
Some steroids have anti-lymphoma effect and
may be used with chemotherapy.
Targeted therapy helps kill lymphoma cells by

stopping key molecules within the cells.
Radiation therapy for mantle cell lymphoma

uses high-energy x-rays to kill cancer cells
within and near to lymph nodes.
A blood stem cell transplant treats lymphoma

by giving a person healthy blood stem cells.
Clinical trials give people access to new tests

and treatments that otherwise can’t usually be
received. These new tests and treatments may
in time be approved by the FDA.

1
4
Chapter title
Treatment guide
initial cap only
x Header
37 Watch and
herewait
initial cap only Guide 1
Guide 1 Guide 2
38 Stage
Guide I2and limited stage II Guide 3
42 Guide 3 stage II and stages III-IV
Extensive
45 Review
x Header here initial cap

4 Treatment guide Watch and wait
Part 4 is a guide to the treatment options

for mantle cell lymphoma. It starts with
explaining a rare “watch and wait”
approach. Then, treatment options by
stage are listed. Your doctor may suggest
other options based on your health and
wishes. Fully discuss your options with
your doctor.
Watch and wait

There are rare times when mantle cell lymphoma
does not need to be treated right away. Instead, a
“watch and wait” approach can be started. Watch
and wait is a period of close observation to see if the
cancer grows and treatment is needed.
Watch and wait may be an option for mantle cell

lymphoma that isn’t causing symptoms. It is more
likely an option if you have a good performance
score and the cancer prognosis is fairly good.
Watch and wait may also be an option for slow-

growing mantle cell lymphoma. As described in
Part 1, these cancers have mutations in the IGHV
region genes and often have low or absent SOX11
levels.

4 Treatment guide Stage I and limited stage II
Stage I and limited stage II More toxic treatments include drugs that can cause
severe side effects. However, the lymphoma doesn’t
Guide 4 lists treatment options for stage I and come back for some time. These drugs are for
limited stage II cancers. These cancers are rare. people younger than age 70, who have good health
They are either above or below the diaphragm. If the other than cancer.
cancer is in more than one cluster of lymph nodes, it
is confined to a small area. More research is needed Supportive care
to learn what treatments are best. During and after cancer treatment, you may be
treated to prevent or control other health conditions.
First-line treatment Such actions are a part of supportive care. Health
Two options are advised by NCCN experts. The first conditions that are a concern for some people
option is immunochemotherapy. Radiation therapy include TLS, reactivated viruses, and other
may follow. The second option is radiation therapy infections. Talk to your doctor about which health
alone. Radiation therapy appears to be a good conditions you may develop as a result of cancer
treatment for limited mantle cell lymphomas. treatment.
For first-line treatment, immunochemotherapy Treatment response

options are divided into two groups. Less toxic At the end of treatment, you will receive an imaging
treatments include drugs that are less likely to harm test to check the results. Imaging may be done with
your body than other drugs. Less toxic treatments CT or PET/CT. If there are no signs of cancer, you
are for people who are: 1) 70 years of age and older; may start follow-up care. If there are signs of cancer,
and 2) younger than 70 years but who have serious read Guide 6 for options.
health problems in addition to cancer. Some doctors
use a younger age to decide who can have more or
fewer toxic drugs.
Guide 4. First-line treatment
What are the options?
• Immunochemotherapy ± radiation therapy

Less toxic treatments More toxic treatments
◦◦ Bendamustine + rituximab ◦◦ CALGB 59909
◦◦ VR-CAP ◦◦ R-Hyper-CVAD
◦◦ Cladribine + rituximab ◦◦ NORDIC
◦◦ RCHOP ◦◦ RCHOP/RDHAP
◦◦ Lenalidomide + rituximab ◦◦ RDHAP
◦◦ Modified R-Hyper-CVAD ◦◦ RCHOP/RICE
• Radiation therapy alone

Primer on first-line drug regimens

Combinations of drugs are almost always used to NORDIC is a 6-cycle regimen. Each cycle is
treat mantle cell lymphomas. These combination 21 days long. Cycle 1 consists of maxi-CHOP
regimens can be complex. Below, the first-line (dose-intensified cyclophosphamide, vincristine,
regimens that are listed in Part 4 are briefly doxorubicin, prednisone) and cycles 3 and 5 consist
described. Ask your treatment team for more of maxi-CHOP with rituximab. Cycles 2, 4, and 6
information. consist of rituximab and high-dose cytarabine.
Bendamustine + rituximab, or BR, is given for up RCHOP is given for up to 6 cycles. Each cycle
to 6 cycles. Each cycle is 28 days long. is 28 days long. RCHOP consists of rituximab,
cyclophosphamide, doxorubicin, vincristine, and
CALGB 59909 is a series of treatments, which prednisone.
includes:
RDHAP is a 4-cycle regimen. Each cycle is
Two or three cycles of R-M-CHOP 21 days long. RDHAP consists of rituximab,
(rituximab, methotrexate, cyclophosphamide, dexamethasone, cisplatin, and cytarabine.
doxorubicin, vincristine, prednisone);
RCHOP/RDHAP is a 6-cycle regimen. Each cycle
One cycle of etoposide, cytarabine, and is 21 days long. Cycles 1 and 2 consist of CHOP
rituximab; (cyclophosphamide, doxorubicin, vincristine,
prednisone). Cycle 3 consists of RCHOP. Cycles
One cycle of high-dose carmustine, 4–6 consist of RDHAP.
etoposide, and cyclophosphamide followed
by autologous stem cell transplant; RCHOP/RICE is a 6- or 7-cycle regimen. Each
cycle is 21 days long. The first 4 cycles consist
Rituximab maintenance weekly for two of RCHOP. You may then receive two or three
weeks. cycles of RICE (rituximab, ifosfamide, carboplatin,
etoposide).
Cladribine + rituximab is given for up to 6 cycles.
Each cycle is 28 days long. R-Hyper-CVAD is an 8-cycle regimen. Each cycle
is 21 days long. Cycles 1, 3, 5, and 7 consist
Lenalidomide + rituximab is given for 12 cycles. of rituximab, cyclophosphamide, vincristine,
Each cycle is 28 days long. doxorubicin, and dexamethasone. Cycles 2,
4, 6, and 8 consist of rituximab, high-dose
Modified R-Hyper-CVAD is given for up to 6 methotrexate, and cytarabine.
cycles. Each cycle is 28 days long. This regimen
is given only to people older than 65 years. No
methotrexate or cytarabine is received. Rituximab
maintenance is received weekly for 4 weeks then
repeated every 6 months for two years.

Follow-up care
Guide 5 lists follow-up care for when there are
no signs of cancer after treatment. This care may
include a medical history, physical exam, imaging,
and blood tests. A test schedule is listed in the Guide.
However, these tests may be done whenever there
are signs or symptoms of cancer. A biopsy is often
needed to confirm there’s cancer. If the cancer
returns (relapse), read Guide 6 for options.
Second-line treatment
Guide 6 lists options for second-line treatment.
These options are used if first-treatment didn’t work.
They are also used if the lymphoma reappears on
tests. Options are based on your prior treatment.
Prior immunotherapy ± radiation therapy

You may have three options. One option is a clinical
trial of new drugs or of a stem cell transplant. The
second option is radiation therapy if you didn’t
receive it before. The third option is a drug regimen
listed in the Guide, or sometimes a regimen for
diffuse large B-cell lymphoma is tried.
Prior radiation therapy only

Your first-line treatment may have consisted
only of radiation therapy. In this case, you may
be able to join a clinical trial. The other option is
immunotherapy. Read Guide 7 for options.

Guide 5. Follow-up care
Type of care How often is this care needed?
Medical history, physical exam, blood • Every 3–6 months for 5 years
counts, lactate dehydrogenase,
metabolic panel, imaging ◦◦ If normal, then repeat every year or when needed
Guide 6. Second-line treatment
Prior first-line treatment What are the options?
• Clinical trial
• Radiation therapy
• Drug treatment
◦◦ Bendamustine ± rituximab
◦◦ Bendamustine, bortezomib, and rituximab

Immunochemotherapy ±
radiation therapy ◦◦ Bortezomib ± rituximab
◦◦ Cladribine + rituximab
◦◦ Ibrutinib
◦◦ Ibrutinib, lenalidomide, rituximab
◦◦ Lenalidomide ± rituximab
◦◦ Venetoclax
• Clinical trial
Radiation therapy only
• Immunochemotherapy (see Guide 7)

4 Treatment guide Extensive stage II and stages III‒IV
Extensive stage II and goal is to fully treat the cancer so there are no cancer
stages III‒IV signs with imaging.
The cancer may be the same or worse after

Guide 7 lists options for extensive stage II and immunochemotherapy. In this case, read Guide 9
stages III and IV. These lymphomas involve a large for options.
area either above or below the diaphragm, are on
both sides of the diaphragm, or have widely spread With blood stem cell transplant
outside the lymphatic system. You may be able to receive a stem cell transplant. In
this case, a clinical trial is an option. Another option
Without blood stem cell transplant is immunochemotherapy followed by an autologous
You may be unable to receive a stem cell transplant. blood stem cell transplant. This option is for people
In this case, a clinical trial is an option. Another who are typically younger than age 70 and who
option is immunochemotherapy that is not too harsh have good health other than cancer. Immunotherapy
on the body. regimens are further explained on page 39
Immunochemotherapy Supportive care

Options are listed in Guide 7. They are for people During and after cancer treatment, you may be
who are 1) 70 years of age and older; and 2) younger treated to prevent or control other health conditions.
than 70 years but who have serious health problems Such actions are a part of supportive care. Health
in addition to cancer. Some doctors use a younger conditions that are a concern for some people
age to decide who should receive these drugs. include TLS, reactivated viruses, and other
These regimens are further explained on page 39. infections. Talk to your doctor about which health
conditions you may develop as a result of cancer
Supportive care treatment.
During and after cancer treatment, you may be
treated to prevent or control other health conditions. Treatment response
Such actions are a part of supportive care. Health CT or PET/CT will be used to assess the results of
conditions that are a concern for some people immunochemotherapy. If the cancer can’t be seen,
include TLS, reactivated viruses, and other you may join a clinical trial or have an autologous
infections. Talk to your doctor about which health stem cell transplant. After the transplant, you may re-
conditions you may develop as a result of cancer start rituximab. It is received every 8 to 12 weeks for
treatment. 2 years or sometimes longer.
Treatment response There may be less cancer after treatment. In this

CT or PET/CT will be used to assess the results of case, a second-line treatment may be received.
immunochemotherapy. If there are no signs of the The goal is to fully treat the cancer so a stem cell
cancer, you may join a clinical trial. Another option transplant is an option.
may be rituximab. It is received every 8 to 12 weeks
for 2 years or sometimes longer. The lymphoma may be the same or worse after
immunochemotherapy. In this case, read Guide 9
There may be less cancer after treatment. In this for options.
case, a second-line treatment may be received. The

Guide 7. First-line treatment

Without blood stem cell transplant
Immunochemotherapy Results Treatment
• Bendamustine + rituximab • Clinical trial

• VR-CAP No signs of cancer
• Consider rituximab maintenance
• Cladribine + rituximab
• RCHOP Cancer looks smaller • Consider second-line treatment
• Lenalidomide + rituximab Cancer looks the
• Read Guide 9
• Modified R-Hyper-CVAD same or larger
With blood stem cell transplant

Immunochemotherapy Results Treatment
• CALGB 59909 • Clinical trial

• R-Hyper-CVAD No signs of cancer
• Autologous stem cell transplant
• NORDIC with rituximab maintenance
• RCHOP/RDHAP Cancer looks smaller • Consider second-line treatment
• RDHAP
Cancer looks the
• RCHOP/RICE • Read Guide 9
same or larger
Guide 8. Follow-up care
Type of care How often is this care needed?
Medical history, physical exam, blood • Every 3–6 months for 5 years
counts, lactate dehydrogenase,
metabolic panel, imaging ◦◦ If normal, then repeat every year or when needed
Follow-up care often needed to confirm there’s cancer. If the cancer

Guide 8 lists follow-up care for when there are returns (relapses), read Guide 9 for options.
no signs of cancer after treatment. This care may
include a medical history, physical exam, imaging,
and blood tests. A test schedule is listed in the Guide.
However, these tests may be done whenever there
are signs or symptoms of lymphoma. A biopsy is

Second-line treatment Guide 9. Second-line treatment

Guide 9 lists options for second-line treatment.
These options are used if first-treatment didn’t work. What are the options?
They are also used if the lymphoma reappears on
tests. The best treatment approach is unknown. More • Clinical trial
research is needed.
• Radiation therapy
One option is to join a clinical trial of new drugs or • Drug treatment

a stem cell transplant. A second option is radiation
◦◦ Bendamustine ± rituximab
therapy if you have not received it before. The
third option is a drug regimen listed in Guide 9. ◦◦ Bendamustine, bortezomib, and rituximab
Sometimes, a regimen for diffuse large B-cell ◦◦ Bortezomib ± rituximab
lymphoma is tried.
◦◦ Cladribine + rituximab
If the lymphoma is fully treated, your doctor may ◦◦ Ibrutinib
suggest a blood stem cell transplant. An allogeneic
transplant is an option. You must be fairly healthy to ◦◦ Ibrutinib, lenalidomide, and rituximab
have this treatment. ◦◦ Lenalidomide ± rituximab
◦◦ Venetoclax

4 Treatment guide Review
Review
Treatment options for mantle cell lymphoma are
based on the cancer stage and your health.
For stage I and limited stage II cancers,

options for first-line treatments are
immunochemotherapy with or without radiation
therapy and radiation therapy alone.
Joining a clinical trial is an option if you have

extensive stage II or stage III or IV cancer.
Besides a clinical trial, most of these cancers
are treated with immunochemotherapy alone. If
your body can endure severe side effects, you
may receive immunochemotherapy followed by
a stem cell transplant.
During and after cancer treatment, you

may be treated to prevent or control other
health conditions. Such actions are a part of
supportive care.
Follow-up care is for cancers that were fully

treated. Your doctor will look for signs of cancer
growth. If tests suggest cancer growth, more
treatment will be needed.
If immunochemotherapy doesn’t work as

planned or the cancer returns, there are
multiple treatment options. You may join a
clinical trial, receive radiation therapy if not
received before, or start a drug regimen that is
advised by NCCN experts.

5
Making treatment decisions
47 It’s your choice
47 Questions to ask your doctors
52 Deciding between options
53 Websites
53 Review

5 Making treatment decisions It’s your choice | Questions to ask
Having cancer is very stressful. While behind your plan but you know your concerns and
absorbing the fact that you have cancer, goals. By working together, you are likely to get a
you have to learn about tests and higher quality of care and be more satisfied. You’ll
treatments. In addition, the time you have likely get the treatment you want, at the place you
to accept a treatment plan feels short. want, and by the doctors you want.
Parts 1 through 4 described the cancer
and treatment options. Part 5 aims to help
you make decisions that are in line with
your beliefs, wishes, and values. Questions to ask your doctors
You may meet with experts from different fields of
medicine. Strive to have helpful talks with each
person. Prepare questions before your visit and ask
It’s your choice questions if the person isn’t clear. You can also take
notes and get copies of your medical records.
The role each person wants in choosing his or her
treatment differs. You may feel uneasy about making It may be helpful to have your spouse, partner, family
treatment decisions. This may be due to a high level member, or a friend with you at these visits. A patient
of stress. It may be hard to hear or know what others advocate or navigator might also be able to come.
are saying. Stress, pain, and drugs can limit your They can help to ask questions and remember what
ability to make good decisions. You may feel uneasy was said. Suggested questions to ask are listed on
because you don’t know much about cancer. You’ve the following pages.
never heard the words used to describe cancer,
tests, or treatments. Likewise, you may think that
your judgment isn’t any better than your doctors’.
Letting others decide which option is best may make

you feel more at ease. But, whom do you want to
make the decisions? You may rely on your doctors
alone to make the right decisions. However, your
doctors may not tell you which option to choose if
you have multiple good options. You can also have
loved ones help. They can gather information, speak
on your behalf, and share in decision-making with
your doctors. Even if others decide which treatment
you will receive, you still have to agree by signing a
consent form.
On the other hand, you may want to take the lead

or share in decision-making. Most patients do. In
shared decision-making, you and your doctors
share information, weigh the options, and agree on
a treatment plan. Your doctors know the science

5 Making treatment decisions Questions to ask
What’s my diagnosis and prognosis?

It’s important to know that there are different types of cancer. Cancer can greatly differ even when people
have a tumor in the same organ. Based on your test results, your doctor can tell you which type of cancer you
have. He or she can also give a prognosis. A prognosis is a prediction of the pattern and outcome of a disease.
Knowing the prognosis may affect what you decide about treatment.
1. Where did the cancer start? In what type of cell? Is this cancer common?
2. What is the cancer stage? Does this stage mean the cancer is advanced?
3. Is this a fast- or slow-growing lymphoma?
4. What tests do you recommend for me?
5. Where will the tests take place? How long will the tests take and will any test hurt?
6. What if I am pregnant?
7. How do I prepare for testing?
8. Should I bring a list of my medications?
9. Should I bring someone with me?
10. How often are these tests wrong?
11. Would you give me a copy of the pathology report and other test results?
12. Who will talk with me about the next steps? When?

What are my options?

There is no single treatment practice that is best for all people. There is often more than one treatment option
along with clinical trial options. Your doctor will review your test results and recommend treatment options.
1. What will happen if I do nothing?
2. Can I just carefully monitor the cancer?
3. Do you consult NCCN recommendations when considering options?
4. Are you suggesting options other than what NCCN recommends? If yes, why?
5. Do your suggested options include clinical trials? Please explain why.
6. How do my age, health, and other factors affect my options? What if I am pregnant?
7. Which option is proven to work best?
8. Which options lack scientific proof?
9. What are the benefits of each option? Does any option offer a cure or long-term cancer control? Are my
chances any better for one option than another? Less time-consuming? Less expensive?
10. What are the risks of each option? What are possible complications? What are the rare and common side
effects? Short-lived and long-lasting side effects? Serious or mild side effects? Other risks?
11. How do you know if treatment is working?
12. What are my options if my treatment stops working?
13. What can be done to prevent or relieve the side effects of treatment?

What does each option require of me?

Many patients consider how each option will practically affect their lives. This information may be important
because you have family, jobs, and other duties to take care of. You also may be concerned about getting the
help you need. If you have more than one option, choosing the option that is the least taxing may be important
to you:
1. Will I have to go to the hospital or elsewhere? How often? How long is each visit?
2. What do I need to think about if I will travel for treatment?
3. Do I have a choice of when to begin treatment? Can I choose the days and times of treatment?
4. How do I prepare for treatment? Do I have to stop taking any of my medicines? Are there foods I will have to
avoid?
5. Should I bring someone with me when I get treated?
6. Will the treatment hurt?
7. How much will the treatment cost me? What does my insurance cover?
8. Will I miss work or school? Will I be able to drive?
9. Is home care after treatment needed? If yes, what type?
10. How soon will I be able to manage my own health?
11. When will I be able to return to my normal activities?

What is your experience?

More and more research is finding that patients treated by more experienced doctors have better results. It is
important to learn if a doctor is an expert in the cancer treatment he or she is offering.
1. Are you board certified? If yes, in what area?
2. How many patients like me have you treated?
3. How many procedures like the one you’re suggesting have you done?
4. Is this treatment a major part of your practice?
5. How many of your patients have had complications?

5 Making treatment decisions Deciding between options
Deciding between options your options. Choosing your cancer treatment is a

very important decision. It can affect your length and
Deciding which option is best can be hard. Doctors quality of life.
from different fields of medicine may have different
opinions on which option is best for you. This can be Support groups
very confusing. Your spouse or partner may disagree Besides talking to health experts, it may help to talk
with which option you want. This can be stressful. to other people who have walked in your shoes.
In some cases, one option hasn’t been shown to At support groups, you can ask questions and
work better than another. Some ways to decide on hear about the experiences of other people with
treatment are discussed next. lymphoma. Find a support group at the websites
listed on page 53.
2nd opinion
The time around deciding a treatment is very Compare benefits and downsides
stressful. People with cancer often want to get Every option has benefits and downsides. Consider
treated as soon as possible. They want to make these when deciding which option is best for you.
their cancer go away before it spreads farther. While Talking to others can help identify benefits and
cancer can’t be ignored, usually there is time to think downsides you haven’t thought of. Scoring each
about and choose which option is best for you. factor from 0 to 10 can also help since some factors
may be more important to you than others.
You may wish to have another doctor review your
test results and suggest a treatment plan. This is
called getting a 2nd opinion. You may completely trust
your doctor, but a 2nd opinion about which option is
best can help.
Copies of the pathology report, a DVD of the

imaging tests, and other test results need to be sent
to the doctor giving the 2nd opinion. Some people
feel uneasy asking for copies from their doctors.
However, a 2nd opinion is a normal part of cancer
care.
When doctors have cancer, most will talk with more

than one doctor before choosing their treatment.
What’s more, some health plans require a 2nd
opinion. If your health plan doesn’t cover the cost
of a 2nd opinion, you have the choice of paying for it
yourself.
If the two opinions are the same, you may feel more
at peace about the treatment you accept to have.
If the two opinions differ, think about getting a 3rd
opinion. A 3rd opinion may help you decide between

5 Making treatment decisions Websites | Review
Websites Review
American Cancer Society Shared decision-making is a process in which
cancer.org/cancer/non-hodgkin-lymphoma you and your doctors plan treatment together.
Leukemia & Lymphoma Society Asking your doctors questions is vital to getting
LLS.org/informationspecialists the information you need to make informed
decisions.
National Cancer Institute (NCI)
cancer.gov/types/lymphoma Getting a 2nd opinion, attending support groups,
and comparing benefits and downsides may
National Coalition for Cancer Survivorship help you decide which treatment is best for you.
canceradvocacy.org/toolbox
NCCN for Patients®

nccn.org/patients
“LLS provides a number of ways

to assist newly diagnosed patients
from peer to peer counseling to
financial assistance to family
support groups.
– Scott
Survivor, Current age 66

Glossary
55 Dictionary
59 Acronyms

Dictionary
Dictionary
allogeneic blood stem cell transplant cancer stage
A cancer treatment that replaces blood stem cells with donor Ratings of tumors that suggest the outlook of the disease.
stem cells which in turn make a new immune system and
attack the lymphoma.
chemotherapy
Drugs that stop the life cycle of cells so they don’t increase
anesthesia in number.
Loss of feeling with or without loss of wakefulness that is
caused by drugs.
chromosome
Strands of genetic material inside of cells.
antibody
A protein made by white blood cells that helps fight off
chyle
A fatty liquid absorbed from the gut into the lymphatic
infection. Also called an immunoglobulin.
system.
antigen
Any substance that activates the immune system.
clinical trial
Research on a test or treatment to assess its safety or how
autologous blood stem cell transplant well it works.
A cancer treatment that destroys cancer cells with high
doses of chemotherapy then rebuilds destroyed bone
colonoscopy
Use of a thin, long tool that is guided into the colon to view
marrow with your own healthy blood stem cells. Also called
or remove tissue.
an HDT/ASCR (high-dose therapy with autologous stem
cell rescue). complete blood count (CBC)
A test of the number of blood cells in a sample.
B symptoms
Fevers, heavy night sweats, and weight loss without dieting comprehensive metabolic panel
caused by B-cell cancers. Tests of up to 14 chemicals in your blood.
B-cell computed tomography (CT)
One of three types of a white blood cell called a lymphocyte. A test that uses x-rays to view body parts.
beta-2 microglobulin contrast
A small protein made by many types of cells. A dye put into your body to make clearer pictures during
imaging tests.
biopsy
Removal of small amounts of tissue or fluid to be tested for deoxyribonucleic acid (DNA)
disease. A chain of chemicals inside cells that contains coded
instructions for making and controlling cells.
bone marrow
Soft, sponge-like tissue in the center of most bones where diagnose
blood cells are made. To identify a disease.
bone marrow aspiration differential
Removal of a small amount of bone marrow that is liquid to Measurement of the different types of white blood cells
test for disease. present in a blood sample.
bone marrow biopsy echocardiogram
Removal of a small amount of solid bone and bone marrow A test that uses sound waves to make pictures of the heart.
to test for disease.

Dictionary
endoscopy immunochemotherapy
Use of a thin, long tool that is guided into the digestive track The use of both chemotherapy and other drugs that trigger a
to view or remove tissue. response from the immune system.
external beam radiation therapy (EBRT) immunohistochemistry (IHC)

Treatment with radiation that is delivered by a machine into A test of cancer cells to find specific cell traits involved in
the body. abnormal cell growth.
fatigue immunomodulator
Severe tiredness despite getting enough sleep that limits A type of drug that modifies some parts of the body’s
one’s ability to function. disease-fighting system.
fertility specialist intensity-modulated radiation therapy (IMRT)

An expert who helps men and women have babies. Radiation therapy that uses small beams of different
strengths based on the thickness of the tissue.
flow cytometry
A test that looks at certain substances on the surface of involved-site radiation therapy (ISRT)
cells to identify the type of cells present. Treatment with high-energy rays (radiation) that is delivered
to lymph nodes and nearby sites with cancer.
fluorescence in situ hybridization (FISH)
A lab test that uses special dyes to look for abnormal karyotype
chromosomes. A test that uses a microscope to examine a cell’s
chromosomes.
four-dimensional computed tomography (4D-CT)
A CT scan that can show the movement of organs. kinase inhibitor
Cancer treatment that stops the transfer of phosphates,
gastrointestinal (GI) tract which blocks growth signals to cancer cells.
The group of organs through which food passes after being
eaten. lactate dehydrogenase
A protein that helps to make energy in cells.
gene
Instructions in cells for making and controlling cells. liver
Organ that removes waste from the blood and helps to
general anesthesia digest food.
A controlled loss of wakefulness from drugs.
local anesthesia
germinal center A controlled loss of feeling in a small area of the body
A short-lived structure that forms within a lymphatic organ in
caused by drugs.
response to germs.
lumbar puncture
human leukocyte antigen (HLA) typing A procedure in which a thin needle is inserted between the
A blood test that finds a person’s unique set of proteins on
bones of the spine to remove a sample of spinal fluid or give
cells.
drugs into the spinal fluid.
image-guided radiation therapy (IGRT) lymph
Radiation therapy that uses imaging tests during treatment
A clear fluid containing white blood cells.
to better target the tumor.
lymph node
imaging test Small groups of special disease-fighting cells located
A test that makes pictures (images) of the inside of the body.
throughout the body.
immune system lymph vessel
The body’s natural defense against infection.
Tube-shaped ducts that carry lymph throughout the body.

Dictionary
lymphatic system radiation therapy

Network in the body that collects and transports a fluid The use of radiation to treat cancer.
(lymph) and fights germs.
regional anesthesia
lymphocyte A type of drug used for short-term loss of feeling or
A type of white blood cell that helps protect the body from awareness in a part of the body without loss of wakefulness.
illness.
sedative
lymphoma A drug that helps a person to relax or go to sleep.
Cancer that begins in white blood cells called lymphocytes
that are within the lymphatic system.
side effect
An unplanned physical or emotional response to treatment.
magnetic resonance imaging (MRI)
A test that uses a magnetic field and radio waves to make
spleen
An organ to the left of the stomach that helps protect the
pictures of the insides of the body.
body from disease.
mantle zone
An area within lymph nodes containing B-cells that can’t
stem cell transplant
A cancer treatment that destroys bone marrow then
make antibodies yet.
replaces it by adding healthy blood stem cells.
medical history
All health events and medications taken to date.
steroid
A drug used to reduce redness, swelling, and pain, but also
monoclonal antibody to kill cancer cells.
Man-made antibodies that attach proteins on cancer cells.
supportive care
multi-gated acquisition (MUGA) scan Treatment for the symptoms or health conditions caused by
A test of the heart that uses radiation to make pictures. cancer or cancer treatment.
natural killer (NK) cell targeted therapy

One of three types of a white blood cell called a lymphocyte. Drugs that stop the growth process that is specific to cancer
cells.
pathologist
A doctor who’s an expert in testing cells to find disease. T-cell
One of three types of a white blood cell called a lymphocyte.
performance status
A rating of one’s ability to do daily activities. three-dimensional conformal radiation therapy
(3D-CRT)
physical exam Radiation therapy that uses beams that match the shape of
A review of the body by a health expert for signs of disease.
the tumor.
positron emission tomography (PET) thymus
A test that uses radioactive material to see the shape and
A gland located behind the breastbone.
function of body parts.
tonsil
positron emission tomography/computed A group of tissue within the throat that contains many white
tomography (PET/CT) blood cells called lymphocytes and fights germs that enter
A test that uses radioactive material and x-rays to view the
the mouth and nose.
shape and function of organs and tissues.
translocation
proton therapy The switching of parts between two chromosomes.
Radiation therapy that uses protons to treat a disease. Also
called hadron therapy.

Dictionary
tumor lysis syndrome

A condition that occurs when many cancer cells die very
quickly and release their contents into the blood, which can
damage the kidneys and other organs.
ultrasound
A test that uses sound waves to take pictures of the inside
of the body.
uric acid
A chemical that is made and released into the blood when
cells and other substances in the body break down.

Acronyms
Acronyms
3D-CRT IFRT
three-dimensional conformal radiation therapy involved-field radiation therapy
4D-CT IGHV
four-dimensional computed tomography immunoglobulin heavy-chain variable
BTK IGRT
Bruton’s tyrosine kinase image-guided radiation therapy
CAM IHC
complementary and alternative medicine immunohistochemistry
CBC IMRT
complete blood count intensity-modulated radiation therapy
CNS ISRT
central nervous system involved-site radiation therapy
CT LDH
computed tomography lactate dehydrogenase
DNA LINAC
deoxyribonucleic acid linear accelerator
EBRT MRI
external beam radiation therapy magnetic resonance imaging
FDA MUGA
Food and Drug Administration multi-gated acquisition
FISH NCCN®
fluorescence in situ hybridization National Comprehensive Cancer Network®
FNA NK cell
fine-needle aspiration natural killer cell
GI PET
gastrointestinal positron emission tomography
GVHD TLS
graft-versus-host disease tumor lysis syndrome
GVT
graft-versus-tumor
HDT/ASCR
high-dose therapy with autologous stem cell rescue
HLA
human leukocyte antigen

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NCCN Panel Members
NCCN Panel Members for

B-cell Lymphomas
Andrew D. Zelenetz, MD, PhD/Chair Thomas M. Habermann, MD Erin D. Snyder, MD
Memorial Sloan Kettering Cancer Center Mayo Clinic Cancer Center University of Alabama at Birmingham
Comprehensive Cancer Center
Leo I. Gordon, MD/Co-Vice Chair Nancy Lee Harris, MD
Robert H. Lurie Comprehensive Cancer Massachusetts General Hospital Lode J. Swinnen, MB, ChB
Center of Northwestern University Cancer Center The Sidney Kimmel Comprehensive
Cancer Center at Johns Hopkins
Jeremy S. Abramson, MD Francisco Hernandez-Ilizaliturri, MD
Massachusetts General Hospital Roswell Park Cancer Institute Julie M. Vose, MD, MBA
Cancer Center Fred & Pamela Buffett Cancer Center
Mark S. Kaminski, MD
Ranjana H. Advani, MD University of Michigan
Stanford Cancer Institute Comprehensive Cancer Center NCCN Staff
C. Babis Andreadis, MD, MSCE Christopher R. Kelsey, MD Mary Dwyer, MS

UCSF Helen Diller Family Duke Cancer Institute Senior Manager Guidelines
Comprehensive Cancer Center
Nadia Khan, MD Hema Sundar, PhD
Nancy Bartlett, MD Oncology Scientist
Fox Chase Cancer Center
Siteman Cancer Center at Barnes- Senior Medical Writer
Jewish Hospital and Washington Susan Krivacic, MPAff
University School of Medicine Consultant
Paolo Caimi, MD Ann S. LaCasce, MD

Case Comprehensive Cancer Center/ Dana-Farber/Brigham and Women’s
University Hospitals Seidman Cancer Center
Cancer Center
and Cleveland Clinic Taussig Cancer Institute
Amitkumar Mehta MD
Julie E. Chang, MD University of Alabama at Birmingham
University of Wisconsin Comprehensive Cancer Center
Carbone Cancer Center
Auayporn Nademanee, MD
Julio C. Chavez, MD City of Hope Comprehensive Cancer Center
Moffitt Cancer Center
Rachel Rabinovitch, MD
Beth Christian, MD University of Colorado Cancer Center
The Ohio State University Comprehensive
Cancer Center - James Cancer Hospital Nishitha Reddy, MD
and Solove Research Institute Vanderbilt-Ingram Cancer Center
Luis E. Fayad, MD Erin Reid, MD

The University of Texas UC San Diego Moores Cancer Center
MD Anderson Cancer Center
Stephen D. Smith, MD
Martha J. Glenn, MD Fred Hutchinson Cancer Research Center/
Huntsman Cancer Institute Seattle Cancer Care Alliance
at the University of Utah
For disclosures, visit www.nccn.org/about/disclosure.aspx.

NCCN Member Institutions
NCCN Member Institutions

Fred & Pamela Buffett Cancer Center The Sidney Kimmel Comprehensive Stanford Cancer Institute
Omaha, Nebraska Cancer Center at Johns Hopkins Stanford, California
800.999.5465 Baltimore, Maryland 877.668.7535
nebraskamed.com/cancer 410.955.8964 cancer.stanford.edu
hopkinskimmelcancercenter.org
Case Comprehensive Cancer Center/ University of Alabama at Birmingham
University Hospitals Seidman Cancer Robert H. Lurie Comprehensive Cancer Comprehensive Cancer Center
Center and Cleveland Clinic Taussig Center of Northwestern University Birmingham, Alabama
Cancer Institute Chicago, Illinois 800.822.0933
Cleveland, Ohio 866.587.4322 www3.ccc.uab.edu
800.641.2422 • UH Seidman Cancer Center cancer.northwestern.edu
uhhospitals.org/seidman UC San Diego Moores Cancer Center
866.223.8100 • CC Taussig Cancer Institute Mayo Clinic Cancer Center La Jolla, California
my.clevelandclinic.org/services/cancer Phoenix/Scottsdale, Arizona 858.657.7000
216.844.8797 • Case CCC Jacksonville, Florida cancer.ucsd.edu
case.edu/cancer Rochester, Minnesota
800.446.2279 • Arizona UCSF Helen Diller Family
City of Hope Comprehensive 904.953.0853 • Florida Comprehensive Cancer Center
Cancer Center 507.538.3270 • Minnesota San Francisco, California
Los Angeles, California mayoclinic.org/departments-centers/mayo- 800.689.8273
800.826.4673 clinic-cancer-center cancer.ucsf.edu
cityofhope.org
Memorial Sloan Kettering University of Colorado Cancer Center
Dana-Farber/Brigham and Cancer Center Aurora, Colorado
Women’s Cancer Center New York, New York 720.848.0300
Massachusetts General Hospital 800.525.2225 coloradocancercenter.org
mskcc.org
Cancer Center
Boston, Massachusetts University of Michigan
877.332.4294 Moffitt Cancer Center Comprehensive Cancer Center
dfbwcc.org Tampa, Florida Ann Arbor, Michigan
massgeneral.org/cancer 800.456.3434 800.865.1125
moffitt.org mcancer.org
Duke Cancer Institute
Durham, North Carolina The Ohio State University The University of Texas
888.275.3853 Comprehensive Cancer Center - MD Anderson Cancer Center
dukecancerinstitute.org James Cancer Hospital and Houston, Texas
Solove Research Institute 800.392.1611
Fox Chase Cancer Center Columbus, Ohio mdanderson.org
Philadelphia, Pennsylvania 800.293.5066
888.369.2427 cancer.osu.edu University of Wisconsin
foxchase.org Carbone Cancer Center
Roswell Park Cancer Institute Madison, Wisconsin
Huntsman Cancer Institute Buffalo, New York 608.265.1700
at the University of Utah 877.275.7724 uwhealth.org/cancer
Salt Lake City, Utah roswellpark.org
877.585.0303 Vanderbilt-Ingram Cancer Center
huntsmancancer.org Siteman Cancer Center at Barnes- Nashville, Tennessee
Jewish Hospital and Washington 800.811.8480
Fred Hutchinson Cancer University School of Medicine vicc.org
Research Center/Seattle Cancer Care St. Louis, Missouri
Alliance 800.600.3606 Yale Cancer Center/
Seattle, Washington siteman.wustl.edu Smilow Cancer Hospital
206.288.7222 • seattlecca.org New Haven, Connecticut
206.667.5000 • fredhutch.org St. Jude Children’s Research Hospital 855.4.SMILOW
The University of Tennessee yalecancercenter.org
Health Science Center
Memphis, Tennessee
888.226.4343 • stjude.org
901.683.0055 • westclinic.com

Index
Index
biopsy 10, 14, 21, 23, 40, 43
blood tests 17, 23, 40, 43
chemotherapy 18, 27–30, 33, 35, 38, 42
clinical trial 33–35, 40–42, 43–45, 49
complementary and alternative medicine 33
complete blood count 16–17
comprehensive metabolic panel 16–17
computed tomography 16, 18–19, 23, 31, 38, 42
diagnosis 10–14, 21, 48
fertility 16, 23
immunohistochemistry 12–13
immunomodulators 26, 35
involved-site radiation therapy 31
lymphatic system 7–9, 14, 42
lymphocyte 8, 14
lymphoma 8–14, 16–18, 20–23, 25–26, 28–30,
33–35, 37–40, 44–45, 48, 53
medical history 15–17, 40–41, 43
NCCN Member Institutions 63
NCCN Panel Members 62
pathology report 14, 52
physical exam 16–17, 40–41, 43
positron emission tomography/computed
tomography 16, 19, 23, 31, 38, 42
radiation therapy 31–32, 35, 38, 40–41, 44–45
stem cell transplant 33, 35, 39–40, 42–45
steroids 29, 35
supportive care 30, 38, 42, 45
targeted therapy 26, 30, 35

Ü
NCCN
GUIDELINES
FOR PATIENTS
®
Mantle Cell
Lymphoma
2017
NCCN Foundation® gratefully acknowledges our Industry supporters AbbVie Inc. and Gilead Sciences Inc. for their support in making
available these NCCN Guidelines for Patients®. NCCN independently develops and distributes the NCCN Guidelines for Patients. Our
industry supporters do not participate in the development of the NCCN Guidelines for Patients and are not responsible for the content
and recommendations contained therein.
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Available online at NCCN.org/patients

LEARNING that you have cancer

The National Comprehensive Cancer Network® (NCCN®) is a not-for-profit

NCCN Guidelines for Patients®:

For more information about the NCCN Guidelines, visit NCCN.org/clinical.asp.

Dorothy A. Shead, MS Laura J. Hanisch, PsyD Rachael Clarke

For more information about NCCN Foundation, visit NCCNFoundation.org.

NCCN Guidelines for Patients®:

The Leukemia & Lymphoma Society (LLS)

NCCN Guidelines for Patients®:

62 NCCN Panel Members

63 NCCN Member Institutions

NCCN Guidelines for Patients®:

Early chapters explain concepts that are repeated

NCCN Guidelines for Patients®:

NCCN Guidelines for Patients®:

You’ve learned that you have or may have Lymphoid tissues

Lymph nodes and other lymphoid tissue are defined

When tissue fluid increases, it drains into vessels.

NCCN Guidelines for Patients®:

The lymphatic system kills

NCCN Guidelines for Patients®:

Hematopathologists spend all of their time looking

NCCN Guidelines for Patients®:

Most human cells contain the

Normal cells increase in number

NCCN Guidelines for Patients®:

The hematopathologist will assess the rate of cancer IHC panel

Mantle cell lymphoma cells have

NCCN Guidelines for Patients®:

Flow cytometry Genetic tests

Copyright © 2017 National Comprehensive Cancer Network® (NCCN®). www.nccn.org

NCCN Guidelines for Patients®:

Tests that detect translocations are a karyotype or Review

Review your pathology report(s) with your doctor. Ask

NCCN Guidelines for Patients®:

NCCN Guidelines for Patients®:

Guide 1. Health care before cancer treatment

Must haves Sometimes useful

Medical history Beta-2 microglobulin

Echocardiogram or MUGA if you will receive certain

Pregnancy test if you can have babies

NCCN Guidelines for Patients®:

Physical exam Blood tests

Enlarged structures Complete blood count with differential

NCCN Guidelines for Patients®:

damaged. Thus, a high level of LDH is a sign of Imaging tests

NCCN Guidelines for Patients®:

The contrast may cause you to feel flushed or get

Pictures of the insides of your body can

For PET, a sugar radiotracer will be injected into

Whole-body PET/CT may be given in addition to or

Copyright © 2017 National Comprehensive Cancer Network® (NCCN®).

NCCN Guidelines for Patients®:

Scopes colonoscope. The picture from the colonoscope will

The colonoscope will be inserted into your anus

little during the test to help your doctor guide the

NCCN Guidelines for Patients®:

Often, these procedures are done at the same time.

A bone marrow exam removes a sample

My initial symptom was an

IMRT (intensity-modulated radiation therapy) is Late effects

Clinical trials Phase IV trials test drugs approved by the U.S.

Phase III trials compare a new drug to a

Targeted therapy helps kill lymphoma cells by

Radiation therapy for mantle cell lymphoma

A blood stem cell transplant treats lymphoma

Clinical trials give people access to new tests