Professional Documents
Culture Documents
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NCCN at u le
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GUIDELINES s/ ey
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FOR PATIENTS
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2017 rv
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This book focuses on the treatment of diffuse large B-cell lymphoma. Key points of
the book are summarized in the NCCN Quick Guide™. NCCN also offers patient
books on multiple myeloma, 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.
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.
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.
© 2017 National Comprehensive Cancer Network, Inc. Based on the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®)
B-cell Lymphomas (Version 5.2017). Posted 9/28/2017.
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
Endorsed by
Contents
6 How to use this book
7 Part 1
DLBCL basics
Explains how and where this lymphoma starts.
14 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 diffuse large B-cell lymphoma.
37 Part 4
Treatment guide
Presents treatment options for first- and second-line treatment.
46 Part 5
Making treatment decisions
Offers tips for choosing the best treatment.
54 Glossary
Dictionary
Acronyms
64 Index
Who should read this book? briefly describes all the types of treatments so you
can understand your options that are listed in Part 4.
Diffuse large B-cell lymphoma is the focus of this Tips for making treatment decisions are presented in
book. It is also known as DLBCL, for short. It is the Part 5.
most common type of non-Hodgkin’s lymphoma.
Lymphatic system Lymph nodes and other lymphoid tissue are defined
by high numbers of lymphocytes. Lymph also has
Before learning about DLBCL (diffuse large B-cell lymphocytes. Lymphocytes are a type of white
lymphoma), it is helpful to know about the lymphatic blood cell. They help fight germs. The three types of
system. It is one of 13 systems of the human body. lymphocytes are NK (natural killer) cells, B-cells, and
It transports fluids to the bloodstream and fights T-cells. Lymphocytes are made in bone marrow then
germs. As such, it supports your blood-flowing are moved by blood to the lymphatic system.
(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.
In the United States most non-Hodgkin’s antigens include viruses, bacteria, chemicals, and
lymphomas—85 out of every 100—are B-cell pollen. Some antigens are formed inside your body
lymphomas. About 10 out of 100 are T-cell like those found on tissue cells. Antibodies attach
lymphomas. A few have unknown cell origin. It is to antigens, which triggers a response from your
now known that most Hodgkin lymphomas are also immune system.
from B-cells. Thus, Hodgkin and non-Hodgkin’s
lymphomas are more related than first thought. Cell of origin
DLBCL is a cancer of B-cells that have been exposed
Diffuse large B-cell lymphoma to antigens. Some DLBCLs start from B-cells that are
DLBCL is a type of non-Hodgkin’s lymphoma. It within the “factories” of your lymphatic organs. These
is a cancer of B-cells. There are many types of factories are called germinal centers. Germinal
B-cells and, thus, many B-cell cancers. B-cells centers are short-lived structures that are formed
differ from one another based on the cell’s stage of in response to an outside antigen. B-cells undergo
development. As B-cells “mature” they change in changes within the germinal centers to prepare
their ability to make antibodies. them for making antibodies. Other DLBCLs start
from B-cells that have been released from germinal
Antibodies are Y-shaped proteins that are made centers.
in response to the presence of antigens. Some
antigens enter your body from outside. Such
Figure 1
Lymphatic system
Illustration Copyright © 2017 Nucleus Medical Media, All rights reserved. www.nucleusinc.com
Mutations Diagnosis
Cells have a control center called the nucleus.
The nucleus contains chromosomes, which are DLBCL is often a fast-growing tumor. The tumor
long strands of DNA (deoxyribonucleic acid) tightly often occurs in lymph nodes within the neck, armpit,
wrapped around proteins. See Figure 2. Within or groin area but may appear elsewhere. Other
DNA are coded instructions for building new cells and common sites are the stomach and gut. When your
controlling how cells behave. These instructions are doctor suspects cancer, testing is needed. The tests
called genes. that are needed to confirm (diagnose) DLBCL 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 DLBCL, an incisional or excisional biopsy is
advised. An incisional biopsy removes only a part
Cancer’s threat of the tumor through a cut made into your body. An
Cancer cells don’t behave like normal cells. First, the excisional biopsy removes the whole tumor. The
mutations cause cancer cells to grow more quickly methods used to do either biopsy depend on where
and live longer than normal cells. Normal cells grow 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 diagnose DLBCL.
damaged. Over time, the lymphoma cells may build You may have cancer even if these biopsies find no
up in tissues and may travel in blood or lymph to 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
DLBCL, the pathologist should be a specialist in
hematopathology.
Figure 2
Genetic material in cells
Illustration Copyright © 2017 Nucleus Medical Media, All rights reserved. www.nucleusinc.com
Figure 3
Normal cell growth vs.
cancer cell growth
Illustration Copyright © 2017 Nucleus Medical Media, All rights reserved. www.nucleusinc.com
The way cancer cells look may help to identify which BCL6, CD3, CD5, CD10, CD20, CD45, IRF4/MUM1,
type of cancer it is. DLBCL gets its name from the Ki-67, and MYC. DLBCL cells often have CD20 and
way it looks. The cancer cells are large and grow CD45 and no CD3.
throughout tissue rather than in clusters.
In some cases, it is helpful to learn the lymphoma
Protein tests subtype. To do so, the panel should also include
For diagnosis, the hematopathologist needs to study ALK, CD30, CD138, cyclin D1, EBER-ISH, HHV8,
the proteins in the cells’ surface (membrane). This is SOX11, and kappa and lambda light chain proteins.
called immunophenotyping. DLBCL has a common Light chain proteins are part of antibodies.
pattern or “signature” of proteins. See Figure 4.
These proteins are also studied to learn if the cancer Flow cytometry
is a GCB (germinal center B-cell) or non-GCB Flow cytometry is a newer method that can also be
subtype. used to assess the surface proteins on lymphoma
cells. This method involves first adding a marker—a
IHC panel light-sensitive dye—to cells. Then, your blood will
An IHC (immunohistochemistry) panel is a test be passed through a flow cytometry machine. The
for these proteins. It involves applying a chemical machine measures surface proteins on thousands of
marker to cells and then looking at them with a cells.
microscope. The IHC panel should test for BCL2,
Figure 4
CD20 protein
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
Doctors plan treatment with many sources Symptoms are a part of your medical history. Some
of information. One of these sources is symptoms of DLBCL are tiredness and a feeling of
tests of your health and the cancer. Part 2 fullness in your belly. This cancer may also cause
describes who should receive which tests “B symptoms.” It’s important that your doctor knows
before treatment. Some of these tests if you have them. These symptoms include fevers,
are repeated during and after treatment. chills, night sweats, and weight loss without dieting.
Besides tests, Part 2 describes other
types of care that are important to receive Some cancers and other health conditions can run in
before cancer treatment. families. Thus, your doctor will ask about the medical
history of your blood relatives. Your doctor may
ask about the health of your siblings, your parents
and their siblings, and your grandparents and their
siblings. Be prepared to tell who in your family has
Medical history had what diseases and at what ages.
Your medical history includes any health events and A medical history is one of the tests needed for
medicines you’ve taken in your life. You will be asked treatment planning. See Guide 1 for a complete
about illnesses, injuries, health conditions, and more. list of care that is recommended prior to treatment.
It may help to make a list of old and new medications Some types of care are for anyone with DLBCL while
while at home to bring to your doctor’s office. others may be useful for some people.
LDH
LDH (lactate dehydrogenase) is a protein that is
in most cells. It gets into your blood when a cell is
damaged. Thus, a high level of LDH is a sign of cell
damage. High levels can be caused by cancer or
other health problems. If related to lymphoma, high
Hepatitis testing
“
Hepatitis B can be an important factor in the
treatment of DLBCL. Hepatitis B can become active
again due to the cancer or some of its treatments.
Thus, tell your treatment team if you’ve ever been
infected with hepatitis. If you’re unsure, ask your
treatment team if you should get tested. Testing may
be needed if you will be treated with a drug called My diagnosis was sudden,
rituximab.
unexpected and life shattering. I am
Uric acid a non-smoker and runner, and had
Some people with DLBCL are at risk for TLS
just completed a ½ marathon before
(tumor lysis syndrome). This syndrome can be life
threatening. It occurs when the waste released by diagnosis. My only symptom was
dead cells is not quickly cleared out of your body. a persistent cough. My tumor was
This results in kidney damage and severe blood
electrolyte disturbances.
10 cm by 14 cm and causing fluid
to back up in my heart and lungs.
TLS can occur among people with DLBCL who are
– Angie
undergoing strong cancer treatments. The cancer
treatment kills many cancer cells. In turn, too much Survivor, Age 52 at diagnosis
tumor cell waste is released within in a short period
of time.
Whole-body PET/CT
PET (positron emission tomography) and CT
(computed tomography) are two types of imaging
tests. When used together, they are called a
PET/CT scan. PET/CT may be done with one or
two machines depending on the cancer center.
Whole-body PET/CT is a test that is needed to plan
treatment for DLBCL.
Stage II is cancer that is in 2 or more clusters Low risk includes scores of 0 and 1.
either above or below your diaphragm.
Low-intermediate risk includes a score of 2.
Stage III is cancer that is in lymph tissue on
both sides of your diaphragm. High-intermediate risk includes a score of 3.
Stage IV is cancer that has widely spread High risk includes scores of 4 or more.
outside the lymphatic system.
There is now more than one version of the IPI. There
When the cancer is staged, other letters are also is an age-adjusted version for people 60 years of age
assigned. The letters “A” and “B” indicate whether and younger. One point is given for: 1) stage III or IV;
B symptoms have been present in the past 6 2) lactate dehydrogenase level above normal; and
months. No symptoms is rated A, and if symptoms 3) performance status score of 2 or greater. A score
are present, B. The letter “E” stands for extranodal of 0 is low risk, a score of 1 is low-intermediate risk, a
disease, which is cancer in sites other than the lymph score of 2 is high-intermediate risk, and a score of 3
nodes. The letter “X” means the cancer is large is high risk.
(>10 cm).
NCCN-IPI
In general, earlier cancer stages have better The NCCN-IPI is a newer version that works better
outcomes. However, doctors define cancer stages than the standard version. One point is given for:
with information from thousands of patients, so a 1) ages 41 to 60; 2) lactate dehydrogenase level
cancer stage gives an average outcome. It may not between 1 and 3; 3) stage III or IV; 4) extranodal
tell the outcome for one person. Some people will disease; and 5) performance status score of 2 or
do better than expected. Others will do worse. Other greater. Two points are given for: 1) ages 61 to 74;
factors not used for staging cancer are also very and 2) lactate dehydrogenase level greater than 3.
important. Such factors include your general health Three points are given for age 75 and older.
and the features of the cancer.
The total number of points is used to assign you to Fertility and pregnancy
one of four risk groups, which are:
Some cancer treatments can limit your ability to have
Low risk includes scores of 0 or 1. a baby. If you want the choice of having babies after
treatment or are unsure, tell your doctors. It may also
Low-intermediate risk includes scores of 2 or 3. help to talk with a fertility specialist before you begin
cancer treatment.
High-intermediate risk includes scores of 4 or 5.
A fertility specialist is an expert in helping people
High risk includes scores of 6 or higher. have babies. The fertility specialist can discuss
with you how to have a baby after treatment. Some
methods of fertility preservation are discussed next.
If you are a woman of childbearing age, important
Heart tests information on pregnancy is also addressed.
Review
Tell your doctor if you have recently had fevers,
night sweats, and weight loss without dieting.
These can be symptoms of DLBCL.
In Part 3, the main treatment types Immunotherapy for DLBCL uses rituximab. It is
for DLBCL are briefly described. an anti-CD20 monoclonal antibody. This human-
Knowing what a treatment is will help made antibody attaches to CD20 on the surface of
you understand your treatment options lymphoma cells. See Figure 8. It works by marking
listed in Part 4. There is more than one the cells for destruction. It may directly kill cells, too.
treatment for DLBCL. Not every person
will receive every treatment described in Rituximab is sold as Rituxan®. It is a liquid that will
this chapter. be slowly injected into your vein (infusion). It often
takes a few hours to receive the full dose. How often
rituximab is received differs among people. Ask your
doctor for more information.
Figure 8
Anti-CD20 monoclonal
antibody
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
“
Angiogenesis inhibitors stop the growth of new blood
vessels that would provide food (nutrients) to the
cancer.
Figure 9
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.
What to expect your doctor how many cycles will be given. Also ask
Chemotherapy used to treat DLBCL is listed in how many days of treatment there are within a cycle.
Guide 2. Most are liquids that are slowly injected
into a vein. Some are a pill that is swallowed. By any Chemotherapy may consist of one or more drugs.
method, the drugs travel in your bloodstream to treat When only one drug is used, it is called a single
cancer throughout your body. Doctors use the term agent. However, not all drugs work the same way,
“systemic” when talking about a cancer treatment for so often more than one drug is used. A combination
the whole body. regimen is the use of two or more chemotherapy
drugs. A steroid, immunotherapy, or both are often
Chemotherapy is given in cycles of treatment days added to chemotherapy.
followed by days of rest. This allows your body to
recover before the next cycle. Cycles vary in length Side effects of chemotherapy
depending on which drugs are used. Often, a cycle is Side effects are unhealthy or unpleasant physical or
2 to 4 weeks long. If you will have chemotherapy, ask emotional responses to treatment. They differ among
Guide 2. Chemotherapy
Methylprednisolone;
A-Methapred
Methylprednisolone
Depo-Medrol®
acetate;
Medrol®
Methylprednisolone
Solu-Medrol®
sodium succinate
Prednisone –
Steroids are a part of some chemotherapy On the surface of some DLBCL cells are proteins
regimens. They are often given on the same days called CD30. Brentuximab consists of a human-
as chemotherapy but only for a few days or a week. made monoclonal antibody, which attaches to CD30.
Prednisone is made in pill form but dexamethasone After attaching, it enters the cells. Then, it releases
and methylprednisolone are made both as a liquid to chemotherapy. By targeting only cells with CD30,
be injected or a pill to be swallowed. fewer normal cells are harmed.
Most side effects of steroids fade away once the Brentuximab vedotin is a treatment option for
drugs are stopped. Common side effects include refractory or relapsed DLBCL that is 1) CD30 positive
feeling hungry, upset stomach, and mood changes. and 2) won’t be treated with a blood stem cell
You may have trouble sleeping. Wounds may be transplant. Read Part 4 for more information.
slow to heal. Swelling of ankles, feet, or hands is also
common. Brentuximab vedotin is sold as Adcetris®. It is slowly
injected into a vein for about 30 minutes. It is often
given every 3 weeks.
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.
Review
Immunotherapy helps your immune system to
attack and destroy cancer cells.
During chemo, I made sure I ate
Lenalidomide is an immunomodulator. It treats (even when not hungry) and drank
DLBCL by modifying your immune system and
plenty of water. After chemo, I had
by other means.
18 radiation treatments. I would
Chemotherapy stops the life cycle of cancer walk my steps until I could do
cells so they can’t increase in number.
8 times up & down (to rebuild my
Some steroids have anti-cancer effects and lung capacity). Then I walked a 5k
may be used with chemotherapy.
during treatment. It was hard but
Brentuximab vedotin is a targeted therapy. my sister was with me.
It attaches to cells with CD30 proteins then – Angie
releases chemotherapy inside.
Survivor
Involved-site radiation therapy kills cancer
within lymph nodes and nearby cells by
damaging DNA.
Part 4 is a guide to the treatment options with RCHOP if you are healthy. If you have heart
for DLBCL. It starts with explaining the problems or are frail, your options include RCEPP,
first treatments received by cancer stage. RCDOP, DA-EPOCH with rituximab, RCEOP, and
If treatment works, the health care for after RGCVP. RGCVP and R-mini-CHOP can be received
treatment is listed. The last section lists if you are older than 80 years of age and have
options for second-line treatment. Your multiple health conditions.
doctor may suggest other options based
on your health and wishes. Fully discuss When chemotherapy is finished, treatment results
your options with your doctor. will be checked. Imaging tests are used. PET/CT
has been found to be very useful for checking
results. Imaging should occur at least 8 weeks after
treatment. Results are used to advise the next steps
in treatment.
First-line treatment
If there are no signs of cancer, there is no need to
Treatment options are partly based on cancer stage. change your treatment plan. Radiation therapy may
Treatment for stages I and II are discussed next. be received.
Treatment for stages III and IV are discussed starting
on page 42. If the cancer looks smaller, a biopsy may be done
to confirm there’s cancer. If no cancer is found,
Stages I & II radiation therapy may be received. If cancer is found
Guide 4 lists options for initial treatment of stages I or a biopsy isn’t done, you have three options. These
and II. These lymphomas are either above or below options are high-dose radiation therapy, a blood stem
the diaphragm. The lymphoma may be in more than cell transplant with or without radiation therapy, and
one cluster of lymph nodes. If so, it is confined to a joining a clinical trial.
small area.
Imaging tests may suggest that the cancer is the
Treatment options are based on the size of the same or larger. In this case, read Guide 8 for
lymphoma. Large (bulky) lymphomas are 7.5 cm or treatment options. Radiation therapy may also be an
larger. Small (non-bulky) lymphomas are smaller option if you can’t receive chemotherapy.
than 7.5 cm. Options 1 and 2 are for lymphomas of
any size. Option 3 is for small lymphomas. Option 2
Option 2 includes treating the cancer with only
Option 1 chemotherapy and rituximab. No radiation will be
The lymphoma will be treated with rituximab-based used. Chemotherapy will be given for 6 cycles.
chemotherapy then by radiation. Chemotherapy will
be given for 6 cycles. If you are unable to receive Some chemotherapy may harm your health.
chemotherapy, involved-site radiation therapy alone Thus, your current health and age will affect which
may be received. chemotherapy you will receive. Start treatment
with RCHOP if you are healthy. If you have heart
Some chemotherapy may harm your health. problems or are frail, your options include RCEPP,
Thus, your current health and age will affect which RCDOP, DA-EPOCH with rituximab, RCEOP, and
chemotherapy you will receive. Start treatment RGCVP. RGCVP and R-mini-CHOP can be received
Option 2
Immunochemotherapy Results Treatment
if you are older than 80 years of age and have checking results. Tests results are used to advise the
multiple health conditions. next steps in treatment.
After 3 to 4 chemotherapy cycles, treatment results If the cancer appears to be gone or smaller, there is
will be assessed. Testing should occur at least 8 no need to change your treatment plan. Complete
weeks after treatment. Imaging tests are used. the 6 cycles of chemotherapy. If the cancer looks
PET/CT has been found to be very useful for the same or larger, a biopsy may be done to confirm
Guide 4 continued
Option 3 for small cancers
Immunochemotherapy Results Treatment
there’s cancer. If no cancer is found, complete the When chemotherapy is finished, treatment results will
6 cycles of chemotherapy. be assessed. Imaging tests are used. PET/CT has
been found to be very useful for checking results.
If cancer is found or a biopsy isn’t done, read Imaging should occur at least 8 weeks after
Guide 8 for treatment options. Radiation therapy treatment.
may be an option if you can’t receive chemotherapy.
Test results are used to advise the next steps in
Option 3 treatment. If there are no signs of cancer, there is
Option 3 maps a third treatment option for small no need to change your treatment plan. Radiation
stage I and II cancers. It includes treating the therapy may be received.
cancer with rituximab-based chemotherapy then
by radiation. Chemotherapy will be given for 3 If the cancer looks smaller, a biopsy may be done
cycles. Some chemotherapy may harm your health. to confirm there’s cancer. If no cancer is found,
Thus, your current health and age will affect which radiation therapy may be received. If cancer is found
chemotherapy you will receive. or a biopsy isn’t done, you have two options. These
options are high-dose radiation therapy and joining a
Start treatment with RCHOP if you are healthy. If clinical trial.
you have heart problems or are frail, your options
include RCEPP, RCDOP, DA-EPOCH with rituximab, Imaging tests may suggest that the cancer is the
RCEOP, and RGCVP. RGCVP and R-mini-CHOP same or larger. In this case, read Guide 8 for
can be received if you are older than 80 years of age treatment options. Radiation therapy may also be an
and have multiple health conditions. option if you can’t receive chemotherapy.
Stages III & IV If the cancer looks the same or larger, a biopsy
Guide 5 maps the initial treatment of stages III may be done to confirm there’s cancer. If no cancer
and IV. These lymphomas are on both sides of your is found, complete the 6 cycles of chemotherapy.
diaphragm. Otherwise, they have widely spread Another option is to join a clinical trial.
outside the lymphatic system.
If cancer is found or a biopsy isn’t done, read
Rituximab-based chemotherapy Guide 8 for treatment options. Radiation therapy
One option includes 6 cycles of rituximab-based may also be an option.
chemotherapy. For select people, radiation to bulky
sites may be received beforehand. A second option is Guide 6 lists options if first-time treatment worked.
to join a clinical trial. If treatment worked, the signs of cancer will be gone
or greatly reduced on tests. One option is to start
Some chemotherapy may harm your health. observation. Observation or “watch-and-wait” is a
Thus, your current health and age will affect which period of testing to see if cancer grows.
chemotherapy you will receive. Start treatment
with RCHOP if you are healthy. If you have heart A second option is radiation therapy. It may be given
problems or are frail, your options include RCEPP, to sites where the cancer had grown large. It may
RCDOP, DA-EPOCH with rituximab, RCEOP, and also be used to treat lymphoma that has spread to
RGCVP. RGCVP and R-mini-CHOP can be received small areas of the bone.
if you are older than 80 years of age and have
multiple health conditions. A third option is maintenance treatment. The goal of
maintenance is to prevent the cancer from returning.
Sometimes DLBCL spreads into the CNS (central Lenalidomide maintenance has been studied among
nervous system). This system includes your brain older people who received RCHOP.
and spinal cord. If this is the case, other cancer drugs
will be added to the treatment described above. Most A fourth option is consolidation treatment with an
CNS disease can be treated with methotrexate. The autologous blood stem cell transplant. The chance
two innermost layers of your brain’s membrane are of the cancer coming back may be high for some
called the leptomeninges. If the cancer has spread to people. The goal of consolidation treatment is to kill
this site, treatment includes both methotrexate and any cancer cells that may remain.
cytarabine.
Treatment results
After 2 to 4 chemotherapy cycles, treatment results Follow-up care
will be assessed. Imaging tests are used. PET/CT
has been found to be very useful for checking Guide 7 provides follow-up care options for when
results. Imaging should occur at least 8 weeks after there are no signs of cancer after treatment. This
treatment. care may include a medical history, physical exam,
imaging, and blood tests. Imaging will involve a CT
Test results are used to advise the next steps in scan of your chest, abdomen, and pelvis. Contrast
treatment. If the cancer appears to be gone or should be used. A biopsy is often needed to confirm
smaller, complete the 6 cycles of chemotherapy. there’s cancer. If the cancer returns (relapses), read
Another option is to join a clinical trial. Guide 8 for options.
Second-line treatment If the cancer looks the same or larger, there are four
options. The first option is to join a clinical trial. The
Guide 8 lists options for second-line treatment. second option is treatment with one of the regimens
These options are used if first-treatment didn’t work. listed in Guide 8 Blood stem cell transplant is not
They are also used if the lymphoma reappears on planned. The third option is radiation therapy to
tests. Your treatment options are based on whether cancer sites to relieve symptoms. Relief of symptoms
you plan to have a blood stem cell transplant. is part of supportive care. The fourth option is
supportive care other than radiation therapy.
Transplant planned
Chemotherapy is first received. Rituximab may be Transplant not planned
added. There is no preferred regimen among those Join a clinical trial if you can. If you can’t, the drug
listed in the Guide. regimens that are listed in the Guide are advised. For
CNS disease, other drugs such as methotrexate will
For CNS disease, other drugs will be added to the be added to the regimen you accept. A third option is
regimen you will receive. Most CNS disease can radiation therapy to cancer sites to relieve symptoms.
be treated with methotrexate. Sometimes rituximab Relief of symptoms is part of supportive care. The
or cytosine arabinoside are also used. The two fourth option is supportive care other than radiation
innermost layers of your brain’s membrane are therapy.
called the leptomeninges. If the cancer has spread to
this site, treatment includes both methotrexate and
cytarabine.
Review
After chemotherapy, treatment results will be
assessed. Imaging tests are used. PET/CT has been Options for initial treatment of DLBCL are
found to be very useful. Imaging should occur at based on the stage of the cancer.
least 8 weeks after treatment. Results are used to
advise the next steps in treatment. Stage I and II cancers are often treated with
6 cycles of immunochemotherapy followed by
If the cancer appears to be gone or smaller, there are radiation therapy. Small cancers may be fully
3 treatment options. The first option is consolidation treated with only 3 cycles. Another option is
treatment with an autologous blood stem cell treating the cancer with only chemotherapy and
transplant. The goal of consolidation treatment is to rituximab.
kill any cancer cells that may remain. The second
and third options are clinical trials and, for some Stage III and IV cancers are often treated with
people, an allogeneic blood stem cell transplant. Ask 6 cycles of immunochemotherapy. A second
your doctor if you can have an allogeneic transplant. option is to join a clinical trial. If 6 cycles of
treatment works, you may be further treated
Radiation therapy may improve the results of a with radiation therapy, lenalidomide, or a blood
transplant. Involved-site radiation therapy before stem cell transplant.
the transplant may be received. In addition, sites
that had cancer may be treated with more radiation Second-line treatment is based on whether a
before or after the transplant. blood stem cell transplant is planned.
• Clinical trial
• Drug treatment
◦◦ Bendamustine ± rituximab
◦◦ CEPP ± rituximab
◦◦ CEOP ± rituximab
◦◦ DA-EPOCH ± rituximab
◦◦ GDP ± rituximab
◦◦ GemOx ± rituximab
◦◦ Lenalidomide ± rituximab
◦◦ Rituximab
• Radiation therapy
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’.
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?
5. Where will the tests take place? How long will the tests take and will any test hurt?
6. What if I am pregnant?
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?
4. Are you suggesting options other than what NCCN recommends? If yes, why?
6. How do my age, health, and other factors affect my options? What if I am pregnant?
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?
13. What can be done to prevent or relieve the side effects of treatment?
1. Will I have to go to the hospital or elsewhere? How often? How long is each visit?
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?
7. How much will the treatment cost me? What does my insurance cover?
3. How many procedures like the one you’re suggesting have you done?
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
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
“
My goal was to run another ½
marathon. I had to have a goal.
This kept depression and despair
away. I took one day at a time. One
year after treatment, I ran and
completed the Niagara’s women’s
half marathon.
– Angie
Survivor
Dictionary
adrenal gland cancer stage
A small organ on top of each kidney that makes hormones. A rating of tumors that suggest the outlook of the disease.
anesthesia chromosome
Loss of feeling with or without loss of wakefulness that is Strands of genetic material inside of cells.
caused by drugs.
chyle
antibody A fatty liquid absorbed from the gut into the lymphatic
A protein made by white blood cells that helps fight off system.
infection. Also called an immunoglobulin.
clinical trial
antigen Research on a test or treatment to assess its safety or how
Any substance that activates the immune system. well it works.
lymphocyte sedative
A type of white blood cell that helps protect the body from A drug that helps a person to relax or go to sleep.
illness.
side effect
lymphoma An unplanned physical or emotional response to treatment.
Cancer that begins in white blood cells called lymphocytes
that are within the lymphatic system.
spleen
An organ to the left of the stomach that helps protect the
magnetic resonance imaging (MRI) body from disease.
A test that uses a magnetic field and radio waves to make
pictures of the insides of the body.
steroid
A drug used to reduce redness, swelling, and pain, but also
medical history to kill cancer cells.
All health events and medications taken to date.
supportive care
monoclonal antibody Treatment for the symptoms or health conditions caused by
Man-made antibodies that attach proteins on cancer cells. cancer or cancer treatment.
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
3D-CRT HLA
three-dimensional conformal radiation therapy human leukocyte antigen
4D-CT IGRT
four-dimensional computed tomography image-guided radiation therapy
CAM IHC
complementary and alternative medicine immunohistochemistry
CBC IMRT
complete blood count intensity-modulated radiation therapy
CNS IPI
central nervous system International Prognostic Index
CT ISRT
computed tomography involved-site radiation therapy
DLBCL LDH
diffuse large B-cell lymphoma 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 cells
fine-needle aspiration natural killer cells
GCB PET
germinal center B-cell 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
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Index
2nd opinion 52 pathology report 13
biopsy 10, 38–40, 42 physical exam 16, 42
blood stem cell transplant 33–34, 38–39, 42–43, positron emission tomography/computed
44–45 tomography (PET/CT) 18, 38–40, 42, 44
blood tests 16–17, 42 radiation therapy 31–32, 38–40, 42–43, 44–45
bone marrow exam 19 shared decision-making 47
cancer stage 20, 21, 38 steroids 30
chemotherapy 27–29, 38–40, 42, 44 supportive care 29, 41, 44–45
clinical trial 35, 38–40, 42–43, 44–45 targeted therapy 30
complementary and alternative medicine
(CAM) 34
complete blood count (CBC) 16
comprehensive metabolic panel 16
computed tomography (CT) 19, 42
diagnosis 10–13
double-hit lymphoma 13
fertility 22
immunohistochemistry (IHC) 12
immunomodulators 26
immunotherapy 25–26
involved-site radiation therapy (ISRT) 31, 44
lumbar puncture 20
lymphatic system 8
lymphocyte 8
lymphoma 8–10
medical history 15, 42
NCCN Member Institutions 63
NCCN panel members 62
observation 42
Diffuse Large
B-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.
PAT-N-1015-0917