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Blood and lymph Unit II > Schowinsky ALL and AML > Flashcards

Schowinsky ALL and AML Flashcards

1
Q

What are acute leukemias

A

clonal neoplastic hematopoietic cells, usually immature, presenting as a rapidly progessing disease

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2
Q

define AML

A

leukemic cells resembling cells of myeloid lineages

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3
Q

define ALL

A

leukemic cells resembling precursor lymphocytes

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4
Q

risk factors for acute leukemia

A

previous chemotherapy, exposure of active marrow to ionizing radiation, tobacco smoke, benzene exposure, and genetic syndromes

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5
Q

T or F: the majority of acute leukemias occur in the apparent absence of risk factors

A

TRUE

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6
Q

T or F: Presenting signs and symptoms usually result from replacement of normal marrow cells by leukemic cells.

A

True: can exhibit S/sx of anemia, thrombocytopenia, and neutropenia

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7
Q

T or F: Thrombotic events, DIC, and direct infiltration of skin, gums, and lymph nodes are very common presenting signs and symptoms of acute leukemias

A

False: these do occur, but more rarely

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8
Q

T or F: neoplams of precursor lymphoid cells manifest as AML, and commonly have a solid mass (lymphoblastic lymphoma)

A

False: manifest as ALL, and solid masses are less common

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9
Q

T or F: 75% of cases of ALL occur in children less than 6 yrs old

A

TRUE

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10
Q

name 3 factors to notice when diagnosing ALL

A

1) nearly always see “packed marrow” full of blast cells. 2) peripheral WBC count can vary greatly (high, normal, or low) 3) determine the blast type by immunophenotyping

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11
Q

what is the basic marker of immaturity

A

CD34

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12
Q

common lymphoblast marker

A

TdT

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13
Q

basic markers for precursor-B cells

A

CD19, CD22

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14
Q

basic markers for precursor-T cells

A

CD3, CD7

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15
Q

T or F: B-ALL accounts for 80-85% of cases of ALL

A

TRUE

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16
Q

T or F: B-ALL usually lacks mature B cell markers like CD20 and surface immunoglobulin

A

TRUE

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17
Q

T or F: B-ALL is the typical ALL of adulthood

A

FALSE

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18
Q

What does t(9:22) indicate

A

“Ph+ ALL”. B-ALL with Philadelphia chromosome, produces a fusion protein of BCR-ABL. Seen in 25% of adult ALL and 2% of childhood ALL. WORST PROGNOSIS OF ANY SUBTYPE ALL

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19
Q

What does translocation 11q23 indicate

A

“MLL” B-ALL more frequently seen in neonates and young infants. Has a poor prognosis

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20
Q

What does t(12:21) indicate

A

“ETV6-RUNX1” 25% of cases of childhood B-ALL. Very favorable prognosis

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21
Q

T or F: T-ALL accounts for around 25% of cases of ALL

A

TRUE

22
Q

T or F: At least 105% of ALL cases are B-ALL and T-ALL

A

FACT!!! 80% B-ALL + 25% T-ALL = 105 fucking %! Where are your gods now?!

23
Q

T or F: T-ALL is less frequent in adolescents and young adults.

A

FALSE

24
Q

T or F: T-ALL often presents with a T-lymphoblastic lymphoma.

A

TRUE: T-LBL can often present as a mediastinal mass

25
Q

What can you say about T-ALL when it comes to WBC and sex?

A

markedly elevated WBC count compared to B-ALL, favors males over females

26
Q

T or F: In general, ALL has a “good prognosis” in children and a worse prognosis in adults

A

TRUE: complete remission rates are >95% vs 60-80% and cure rates are at 80% vs <50% in kids and adults respectively

27
Q

Give 2 factors of better prognosis in ALL with reference to age and disease

A

school age (2-10 yo), B-lymphoblastic hyperdiploidy (51-65)

28
Q

Give some factors of worse prognosis in ALL with reference to age and disease

A

Infants (10 yo), Very high WBC, T-lymphoblastic hypodiploidy (<46), slow response to Rx, Min. Residual disease

29
Q

T or F: AML is a more heterogeneous disease than ALL and is typically seen in adults

A

TRUE

30
Q

What is the big marker for diagnosing AML for most cases?

A

> 20% myeloblasts in the marrow and/or peripheral blood

31
Q

basic markers for the common myeloid lineage

A

CD117(C-kit) and myeloperoxidase

32
Q

What is one hystological feature that can help identify myeloblasts sometimes?

A

Auer rods

33
Q

T or F: some cytogenetic abnormalities allow for diagnosis of AML regardless of blast count

A

TRUE

34
Q

What does t(8:21) indicate

A

“RUNX1-RUNX1T1” 5% of AML cases, younger patients, “AML with maturation” some mature neutrophil production occurs, relatively good prognosis, diagnostic regardless of blast count

35
Q

What does inv(16) or t(16:16)

A

“CBFB-MYH11” 5-10% of AML cases, younger patients, “baso eso” abnormality, typically have myelomonocytic leukemia (myeloblasts and monocytes), relatively good prognosis

36
Q

What does t(15:17) indicate

A

“PML-RARA” acute promyelocytic leukemia (APL), also called M3-AML, hypergranular morphology resembling normal promyelocytes, may see multiple Auer rods, diagnostic of AML regardless of count. Good prognosis if caught early

37
Q

what does the RARA gene do?

A

it is a retinoic acid receptor. PML-RARA is a poorly functioning retinoic acid receptor. Proper function required for differentiation past promyelocyte stage

38
Q

How do we treat APL?

A

supraphysiologic doses of all-trans retinoic acid (ATRA), do not require traditional induction chemo. Can be treated with ATRA and arsenic salts (little morbidity and almost no mortality)

39
Q

What is something really bad that is associated with APL?

A

DIC

40
Q

what does t(1:22) indicate?

A

“RBM15-MKL1”, Megakaryoblastic differentiation, seen in infants with Down syndrome, relatively good prognosis

41
Q

What does 11q23 indicate?

A

“MLL” multiple possible partner genes, show some degree of monocytic differentiation, POOR prognosis

42
Q

Describe therapy related AML (t-AML)

A

AML arising secondary to DNA damage from a prior treatment, 10-20% of AML, VERY BAD PROGNOSIS

43
Q

AML, Not Otherwise Specified (NOS)

A

cases lacking recurrent cytogenetic findings, no known therapy, defined by morphology and immunophenotyping

44
Q

What subtype of AML NOS has myeloblast cells

A

Undifferentiated, minimally differentiated or with maturation

45
Q

What subtype of AML NOS has myeloblast and monocyte cells

A

Myelomonocytic

46
Q

What subtype of AML NOS has monoblast, promonocyte, and/or monocyte cells

A

monoblastic or monocytic

47
Q

What is seen with monoblastic or monocytic AML NOS

A

Leukemic cells often causes lesions in skin and gums

48
Q

What subtype of AML NOS has Megakaryoblasts cells

A

Megakaryoblastic

49
Q

What is seen with Megakaryoblastic AML NOS

A

often associated with significant marrow fibrosis

50
Q

What molecular abnormality has poor prognosis

A

FTL3 Internal Tandem Duplication (ITD)

51
Q

What 2 molecular abnormalities have a good prognosis

A

Nucleophosmin-1 mutation (NPM1) and CEBPA Mutation. This is assuming there is no FLT3 ITD

52
Q

T or F: patients that are healthy enough can have hematopoietic stem cell transplant (SCT).

A

TRUE: Therapy of choice for younger patients, high risk disease, and relapsed disease

Blood and lymph Unit II (6 decks)

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