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Path U2 > CH6 - 2) Acute Leukemia > Flashcards

CH6 - 2) Acute Leukemia Flashcards

1
Q

What is acute leukemia?

A

Neoplastic proliferation of blasts; defined as the accumulation of >20% blasts in the bone marrow.

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2
Q

What is the acute presentation for acute leukemia?

A

Anemia (fatigue), thrombocytopenia (bleeding), or neutropenia (infection)

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3
Q

What is the reason for the acute presentation of acute leukemia?

A

Increased blasts crowd-out normal hematopoiesis,

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4
Q

In acute leukemia, why is there a high WBC count?

A

Blasts usually enter the blood stream, resulting in a high WBC count

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5
Q

Describe blasts.

A

they are large, immature cells, often with punched out nucleoli

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6
Q

What is acute leukemia subdivided into?

A

acute lymphoblastic leukemia (ALL) or acute myelogenous leukemia (AML)

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7
Q

The subdivision of acute leukemia is based on what?

A

the phenotype of the blasts.

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8
Q

What is acute lymphoblastic leukemia?

A

Neoplastic accumulation of lymphoblasts (> 20%) in the bone marrow

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9
Q

Why is TdT useful in characterizing lymphoblasts?

A

It is absent in myeloid blasts and mature lymphocytes.

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10
Q

Acute lymphoblastic leukemia most commonly arises in whom?

A

children

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11
Q

What is acute lymphoblastic leukemia associated with and when does it usually arise?

A

Down syndrome (usually arises after the age of 5 years)

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12
Q

Acute lymphoblastic leukemia is subclassified into? What is it based on?

A

B-ALL and T-ALL based on surface markers

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13
Q

What is the most common type of ALL?

A

B-ALL

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14
Q

What is B-ALL usually characterized by?

A

lymphoblasts (TdT+) that express CD10, CD19, and CD20.

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15
Q

What is the treatment for B-ALL?

A

Excellent response to chemotherapy; requires prophylaxis to scrotum and CSf

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16
Q

What is the prognosis B-ALL based on?

A

It is based on cytogenetic abnormalities,

17
Q

Which B-ALL has a good prognosis and is more commonly seen in children?

A

t(12;21)

18
Q

Which B-ALL has a poor prognosis and in what population is it seen?

A

t(9;22) has a poor prognosis; more commonly seen in adults (Philadelphia+ALL)

19
Q

What is T-ALL characterized by?

A

lymphoblasts (TdT+) that express markers ranging from CD2 to CDH (e.g., CD3, CD4, CD7). The blasts do not express CD10.

20
Q

How does T-ALL usually present?

A

Usually presents in teenagers as a mediastinal (thymic) mass (called acute lymphoblastic lymphoma because the malignant cells form a mass)

21
Q

What is acute myeloid leukemia?

A

Neoplastic accumulation of myeloblasts (> 20%) in the bone marrow

22
Q

How are myeloblasts usually characterized?

A

by positive cytoplasmic staining for myeloperoxidase (MPO)

23
Q

What may be seen in the characterization of myeloblasts?

A

Crystal aggregates of MPO may be seen as Auer rods

24
Q

In whom does acute myeloid leukemia most commonly arise?

A

older adults (average age is 50-60 years)

25
Q

What is the subclassification of AML based on?

A

cytogenetic abnormalities, lineage of myeloblasts, and surface markers.

26
Q

What are the high-yield subtypes?

A

Acute promyelocytic leukemia, Acute monocytic leukemia and acute megakaryoblastic leukemia

27
Q

What is acute promyelocytic leukemia characterized by?

A

t(15;17), which involves translocation of the retinoic acid receptor (RAR) on chromosome 17 to chromosome 15

28
Q

What is the effect of RAR disruption?

A

It blocks maturation and promyelocytes (blasts) accumulate

29
Q

How does acute promyelocytic leukemia lead to increased risk for DIC?

A

Abnormal promyelocytes contain numerous primary granules that increase the risk for DIC

30
Q

What is the treatment for acute promyelocytic leukemia?

A

with all-trans retinoic acid (ATRA, a vitamin A derivative)

31
Q

How does ATRA work?

A

It binds the altered receptor and causes the blasts to mature (and eventually die)

32
Q

What is acute monocytic leukemia?

A

Proliferation of monoblasts; usually lack MPO

33
Q

In acute monocytic leukemia what do blasts characteristically do?

A

infiltrate gums

34
Q

What is acute megakaryoblastic leukemia?

A

Proliferation of megakaryoblasts; lack MPO

35
Q

What is acute monocytic leukemia associated with?

A

Down syndrome (usually arises before the age of 5)

36
Q

What is myelodysplastic syndrome?

A

acute myeloid leukemia may also arise from pre-existing dysplasia = myelodysplastic syndromes, especially with prior exposure to alkylating agents or radiotherapy

37
Q

What do myelodysplastic syndromes usually present with?

A

cytopenias, hypercellular bone marrow, abnormal maturation of cells, and increased blasts < 20%

38
Q

With myelodysplastic syndromes resulting in acute myeloid dysplastic syndrome what do most patients die from?

A

Most patients die from infection or bleeding, though some progress to acute leukemia.

Path U2 (25 decks)

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