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01 Hematology > Acute Leukemia > Flashcards

Acute Leukemia Flashcards

1
Q

What is the definition of leukemia?

A

A progressive, malignant disease of the blood-forming organs, marked by distorted proliferation and development of WBCs and their precursors; primarily involving the bone marrow and blood

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2
Q

What is the major difference between leukemia and lymphoma?

A

Site of involvement

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3
Q

What do acute and chronic mean when in reference to leukemias or lymphomas?

A

Acute indicates that the neoplasm is of immature cells while chronic neoplasms are of mature cells

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4
Q

Why is acute leukemia bad?

A

There is a block in maturation of a cell lineage in the bone marrow and proliferation of those immature cells accompanied by insuficient apoptosis

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5
Q

What are the differences between acute leukemia an myelodysplasia?

A

With acute leukemia there is a block in maturation, increased proliferation with decreased apoptosis; in myelodysplasis there is also ineffective maturation but there is increased apoptosis within the bone marrow

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6
Q

What is the most common presenting sign of leukemia?

A

Peripheral cytopenia

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7
Q

What are the clinical findings associated with leukemias?

A

Peripheral cytopenia, splenomegaly, hepatomegaly, lymphadenopathy, systemic (“B”) symptoms (fatigue, fever, malaise, weight loss), and metabolic alterations

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8
Q

What antigens are used for immunophenotyping myeloid differentiation?

A

CD33, 13, 14, 64, myeloperoxidase

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9
Q

What antigens are used for immunophenotyping T-cell differentiation?

A

CD3, 4, 5, 7, 8

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10
Q

What antigens are used for immunophenotyping B-cell differentiation?

A

CD19, 20, 22, 79a

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11
Q

What antigens are used for immunophenotyping immature cells?

A

CD34, 117, TdT

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12
Q

What is the threshold that differentiates Acute from Chronic leukemia?

A

Greater than 20% of blasts in the bone marrow or peripheral blood indicates a significant block in maturation and an acute process

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13
Q

What are two basic subcategories of Acute Leukemia?

A

Acute Myelogenous Leukemia and Acute Lymphocytic Leukemia

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14
Q

In what kind of acute leukemia may auer rods be found?

A

Myelogenous

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15
Q

What are auer rods?

A

Aggregates of MPO

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16
Q

What are the two classes of mutations that must be acquired for development of acute myelogenous leukemia?

A

Class I (Increased proliferation or survival) and Class II (Impaired differentiation or apoptosis)

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17
Q

What are the subclassifications of AML?

A

AML w/ recurrant cytogenetic abnormalities; AML w/ myelodysplasia-related changes; Myeloid sarcoma; Myeloid proliferation related to Down Syndrome; Blastic plasmacystoid dendritic cell neoplasm; AML not otherwise specified

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18
Q

What chromosomal translocations are associated with a good AML prognosis?

A

t(8;21), t(15;17), inv(16)

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19
Q

What chromosomal translocations are associated with a poor AML prognosis?

A

11q23, t(6;9), inv(3), t(1;22)

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20
Q

Are FLT3 mutations generally associated with a better or poorer prognosis?

A

Poor

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21
Q

What cytogenetic abnormality is associated with Acute Promyelocytic Leukemia?

A

t(15;17)

22
Q

What are the 2 distinct morphological variants of APL?

A

Classic variant- abundant prominent cytoplasmic granules, bundles of auer rods, low WBC count, wt-FLT3; and Microgranular variant- lack prominent cytoplasmic granuels and few auer rods with high WBC counts, more likely to have FLT3 mutation

23
Q

Why is APL a medical emergency?

A

There is a high risk of developing DIC

24
Q

What APL translocation renders cells resistant to all-trans-retinoic acid?

A

Partnering with Ch. 11 (PZLF/ZBTB16)

25
Q

How does all-trans-retinoic acid work in the treatment of acute promyelocytic leukemia?

A

It overcomes the maturatio nblock at the promyelocyte stage

26
Q

How does APL increase risk of developing DIC?

A

It increases expression of fibrinolytic annexin II

27
Q

What is the characteristic morphology of AML with inv(16)?

A

Eo-basos; immature cells with chunky granules that look like a combinatino of eosinophils and basophils and are CAE+

28
Q

MLL abnormalities found at 11q23 can be associated with therapy-related AMLs, especially patients treated with what kind of drug?

A

Topoisomerase II inhibitors

29
Q

AML associated with MLL alterations are associated with a good or poor prognosis?

A

Poor

30
Q

What is characteristic of the morphology of AML with t(6;9)?

A

Basophilia

31
Q

What morphology is associated with AML with inv(3)?

A

Increased # of small megakaryocytes in bone marrow and thrombocytopenia

32
Q

What cytogenetic abnormality is associated with Down Syndrome-associated AML?

A

t(1:22)

33
Q

What is Transient Abnormal Myelopoiesis?

A

Condition found in 10% of Down Syndrome babies that originates in utero and present within 3 months of life, but most will clear the blasts and this is just a transient process but about 19% of children with TAM progress to AML M7

34
Q

True or False: DS pts with M7 AML have a worse prognosis than non-DS AML M7 pts.

A

False

35
Q

What is the prognosis of AML with normal cytogenetics?

A

intermediate prognosis

36
Q

Which mutations are associated with a good prognosis in AML with normal cytogenetics?

A

NPM1 and CEBPA

37
Q

Which mutations are associated with a poor prognosis in AML with normal cytogenetics?

A

FLT3, high ERG, high BAALC, MLL-PTD

38
Q

What is FLT3?

A

TYrosine kinase related to PDGFR

39
Q

What is NPM1?

A

Protein that shuttle between the nucleus and cytoplasm

40
Q

What percentage of AML cases have both NPM1 and FLT3-ITD?

A

~40%

41
Q

How can AML vs ALL morphology be distinguished?

A

Lymphoblasts are smaller than non-lymphoblasts and have very little cytoplasm w/ no prominent nucleoli or auer rods

42
Q

What is the most important determinant of the biology and prognosis of ALL?

A

Underlying genetic abnormality

43
Q

What are the two major WHO categories of ALL?

A

Precursor B lymphoblastic leukemia and Precursor T lymphoblastic leukemia

44
Q

If ALL presents with lymphomatous and leukemic components what threshold of blasts must be present for a leukemic diagnosis?

A

> 25% blasts in the blood or BM defines the condition as leukemia

45
Q

What cytogenetic abnormalities associated with ALL have a good prognosis?

A

t(12:21), hyperdiploidy, 6q-, t(5;14)

46
Q

What cytogenetic abnormalities associated with ALL have a poor prognosis?

A

t(9;22), t(1;19), t(4;11), hypodiploidy

47
Q

What are indicators of poor T-ALL prognosis?

A

LY1, Tal1

48
Q

What are indicator of good T-AL prognosis?

A

HOX11

49
Q

When assigning more than one lineage to a single blast population what are the requirements for assigning a myeloid lineage?

A

Must have MPO OR at least 2 of NSE, CD11c, CD14, CD64, lysozyme

50
Q

When assigning more than one lineage to a single blast population what are the requirements for assigning a T lineage?

A

Cytoplasmic CD3 or Surface CD3

51
Q

When assigning more than one lineage to a single blast population what are the requirements for assigning a B lineage?

A

Strong CD19 with at least one other (strong) CD79a, CD22, CD10 OR Weak CD19 with 2 other strong

01 Hematology (16 decks)

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