Acute Leukemia

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LEC.

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Dr. Bassam M. H.

Leukemia

Group of disorders characterized by proliferation of abnormal cells which


accumulate in the bone marrow.

These cause symptoms by:

1. Interfere with the production of normal blood cells.


2. Infiltration of organs.

Classification

1. Acute AML,ALL
2. Chronic CML,CLL

Definition
The acute leukemias are a heterogeneous group of neoplasms arising
from transformation of uncommitted or partially committed
hematopoietic stem cells

AL progresses rapidly and is typically fatal within weeks or months if left


untreated.

Pathophysiology

In normal hematopoiesis, the blast is an immature precursor of white


blood cells; a normal blast will gradually mature into a mature white
blood cell. However, in AL, a single blast accumulates genetic changes
which "freeze" the cell in its immature state and prevent differentiation.
Such a mutation alone does not cause leukemia; however, when such a
"differentiation arrest" is combined with other mutations which disrupt
genes controlling proliferation, the result is the uncontrolled growth of an
immature clone of cells, leading to the clinical entity of AL

The clinical signs and symptoms of AL result from the fact that, as the
leukemic clone of cells grows, it tends to displace or interfere with the
development of normal blood cells in the bone marrow. This leads to
neutropenia, anemia, and thrombocytopenia

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Signs and symptoms

1. Infections
2. anemia
3. bleeding tendency
4. bone pain
5. Enlargement of the spleen
6. leukemia may be discovered incidentally during a routine blood
test.

Investigations and diagnosis


1. PBF.
2. Bone marrow aspirate & biopsy.
3. Cytochemistry
4. Immunophenotyping of blood or marrow blasts
5. Bone marrow cytogenetics.

AML

Epidemiology of disease

AML is the most common acute leukemia affecting adults It can occur at
all ages but has its peak incidence in the seventh decade

Risk factors for developing AML

1. "Pre-leukemic" blood disorders such as myelodysplastic or


myeloproliferative
2. Exposure to anti-cancer chemotherapy, in particular alkylating
agents,
3. Ionizing radiation exposure can increase the risk of AML
4. Several congenital conditions may increase the risk of leukemia;
the most common is probably Down syndrome,

French-American-British classification

The eight FAB subtypes are

M0 minimally differentiated acute myeloblastic leukemia

M1 (acute myeloblastic leukemia, without maturation)

M2 (acute myeloblastic leukemia, with granulocytic maturation)


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M3 (promyelocytic), or acute promyelocytic leukemia (APL)

M4 (acute myelomonocytic leukemia)

M4eo (myelomonocytic together with bone marrow eosinophilia)

M5 acute monoblastic leukemia (M5a) or acute monocytic leukemia


(M5b)

M6 (acute erythroid leukemias), including erythroleukemia (M6a) and


very rare pure erythroid leukemia (M6b)

M7 (acute megakaryoblastic leukemia)

Prognosis

50% of children and young adult may expect long term cure.

Acute lymphoblastic leukemia (ALL)

ALL is most common in childhood and young adulthood with a peak


incidence at 4-5 years of age, and another peak in old age.

The FAB classification

 ALL-L1: small uniform cells


 ALL-L2: large varied cells
 ALL-L3: large varied cells with vacuoles (bubble-like features)

Prognosis

The overall cure rate in children is 85%, and about 50% of adults have
long-term disease-free survival.

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