Heme Onc [LEUKEMIA]

Introduction
In dealing with Leukemias we must consider whether they’re
acute (undifferentiated, aggressive) or chronic (differentiated,
indolent). The acute leukemia patients are going to be SICK Asx WBC on
routine labs
(fever, night sweats, bleeding, and infection). It’s a product of ↑↑WBC (60-100)
useless, immature cells crowding out effective cell lines, creating
a pancytopenia. Conversely, Chronic leukemia will be
asymptomatic and found on a routine screen for something else Differential
(unless very late stage). Patients present with an enormous Polys Lymphocytes
number of leukocytes. Which line gets elevated is dependent on
the type of cancer. Myelogenous is Neutrophils, while CML CLL
Lymphocytic is Lymphocytes. In all cases the first test will be a
Imatinib Ø or
smear to rule out acute disease (the presence of blasts). Then, a
SCT
differential is done to rule out chronic disease. Definitive
BM
diagnosis is made with a bone marrow biopsy.

1. Acute Myelogenous Leukemia

This is a disease of immature (acute) neutrophils (myelogenous) Cytogenetics
Prognosis
cancer in the blood (leukemia). It can arise de novo after
Confirmatory Dx
exposure to radiation, benzene, or chemo, or be a transformation
(so-called “blast crisis”) from other marrow cancers (CML,
MDS). The symptoms of bleeding, bruising, petechiae, pallor
and fever set in rapidly. CBC is of no use as all values could be ↑
or ↓. What gives the diagnosis away is seeing blasts on a
peripheral smear. To confirm the diagnosis a Bone Marrow Fever, Bone Pain,
Biopsy showing >20%Blasts is required, as well as cytogenetic Infxn, Petechiae,
analysis showing neutrophils (myeloperoxidase). A special form Bleeding, Anemia
of AML, the M3 type (Promyelocytic), is diagnosed by the
presence of Auer Rods. Treatment with chemotherapy
Smear
(idarubicin + Ara-C) can push AML into remission. M3 is
treated with Vitamin A, which induces development out of the Polys Lymphocytes
blast phase by all-trans retinoic acid. >20% blasts on peripheral
AML ALL
blood also makes the diagnosis.
CHEMO CHEMO
2. Acute Lymphoid Leukemia M3: VitA PPx CNS
This is a disease of immature (acute) lymphocytes (lymphoid) BM
cancer in the blood (Leukemia). It’s often found in the pediatric
patient who presents with bleeding and bone pain. As in AML,
look at the smear for blasts then get a Bone Marrow Biopsy to Cytogenetics
confirm >20% blasts and cytogenics. Like AML, it’s treated with Prognosis
Confirmatory Dix
chemo (cyclophosphamide, doxorubicin, vincristine, and
methotrexate) with a fairly decent sustained remission (90%)
and poor cure rate (50%). Consider doing intrathecal ppx
chemo-radiation with Ara-C or Methotrexate, because the
CNS is a sheltered region for ALL to hide while undergoing
therapy for systemic blood and marrow cancer. >20% blasts on
peripheral blood also makes the diagnosis.

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 Heme Onc [LEUKEMIA]

3. Chronic Myelogenous Leukemia

This is a disease of matured (chronic) neutrophils
(Myelogenous) cancer in the blood (leukemia). It’s associated
with the Philadelphia chromosome – a t(9,22) translocation with
overactive activity of a tyrosine kinase BCR-ABL. It presents as
an elevated white count with an abnormal percentage of
neutrophils (>60 WBC, >90% PMNs). Once the diagnosis is
made confirm with a Bone Marrow Biopsy. Revolutionary
therapy with the tyrosine-kinase inhibitor Imatinib has
prolonged survival and delayed the blast crisis. Newer tyrosine-
kinase inhibitors have been used in imatinib-refractory cancers.
However, inevitably this cancer becomes resistant, progresses to
AML, and the patient ultimately succumbs.

4. Chronic Lymphoid Leukemia

This is a disease of mature (chronic) lymphocytes (lymphoid)
cancer in the blood (leukemia). It occurs in old men most
commonly presenting as an asymptomatic ↑ in WBC. A diff will
show an absolute lymphocyte count >50. You might see smudge
cells (artificial rupture of fragile cells during smear preparation)
on smear, but it’s the diff and subsequent bone marrow biopsy
that defines the disease. The average survival is about ten years.
If they’re old do nothing; they’re more likely to die with it than
from it. If they become symptomatic, treat with chemotherapy:
fludarabine or rituximab-based. If the patient is young (<65)
and there’s a donor go ahead and perform a stem cell transplant.

Disease Patient Age Cell 1st Test Best Test Treatment Special
Fever, Ara-C
Bleeding, 7 Lymphoid Smear BM Bx MTX CNS PPx
Petechiae, >20% Blasts Cyclophosphamide
Acute Infection, Doxyrubicin
Pallor
Bruising 67 Myelogenous Smear BM Bx Auer Rods/M3 = Vit Auer Rods
Bone Pain (Neutrophils) >20% Blasts A
Idarubicin + Ara-C

BM Bx
47 Myelogenous Diff Philadelphia Imatinib Blast Crisis
↑White (Neutrophils) Chromosome
Chronic Count, t(9,22)
Found on BCR-ABL
routine screen If old or Ø Donor = Ø
87 Lymphoid Diff BM Bx If old and symptomatic = Chemo
If young and donor = BM Transplant

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