Schowinsky ALL and AML

Question 1

What are acute leukemias

Answer 1

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

Question 2

define AML

Answer 2

leukemic cells resembling cells of myeloid lineages

Question 3

define ALL

Answer 3

leukemic cells resembling precursor lymphocytes

Question 4

risk factors for acute leukemia

Answer 4

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

Question 5

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

Answer 5

TRUE

Question 6

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

Answer 6

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

Question 7

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

Answer 7

False: these do occur, but more rarely

Question 8

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

Answer 8

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

Question 9

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

Answer 9

TRUE

Question 10

name 3 factors to notice when diagnosing ALL

Answer 10

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

Question 11

what is the basic marker of immaturity

Answer 11

CD34

Question 12

common lymphoblast marker

Answer 12

TdT

Question 13

basic markers for precursor-B cells

Answer 13

CD19, CD22

Question 14

basic markers for precursor-T cells

Answer 14

CD3, CD7

Question 15

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

Answer 15

TRUE

Question 16

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

Answer 16

TRUE

Question 17

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

Answer 17

FALSE

Question 18

What does t(9:22) indicate

Answer 18

“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

Question 19

What does translocation 11q23 indicate

Answer 19

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

Question 20

What does t(12:21) indicate

Answer 20

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

Question 21

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

Answer 21

TRUE

Question 22

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

Answer 22

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

Question 23

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

Answer 23

FALSE

Question 24

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

Answer 24

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

Question 25

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

Answer 25

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

Question 26

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

Answer 26

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

Question 27

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

Answer 27

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

Question 28

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

Answer 28

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

Question 29

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

Answer 29

TRUE

Question 30

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

Answer 30

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

Question 31

basic markers for the common myeloid lineage

Answer 31

CD117(C-kit) and myeloperoxidase

Question 32

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

Answer 32

Auer rods

Question 33

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

Answer 33

TRUE

Question 34

What does t(8:21) indicate

Answer 34

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

Question 35

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

Answer 35

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

Question 36

What does t(15:17) indicate

Answer 36

"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

Question 37

what does the RARA gene do?

Answer 37

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

Question 38

How do we treat APL?

Answer 38

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)

Question 39

What is something really bad that is associated with APL?

Answer 39

DIC

Question 40

what does t(1:22) indicate?

Answer 40

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

Question 41

What does 11q23 indicate?

Answer 41

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

Question 42

Describe therapy related AML (t-AML)

Answer 42

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

Question 43

AML, Not Otherwise Specified (NOS)

Answer 43

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

Question 44

What subtype of AML NOS has myeloblast cells

Answer 44

Undifferentiated, minimally differentiated or with maturation

Question 45

What subtype of AML NOS has myeloblast and monocyte cells

Answer 45

Myelomonocytic

Question 46

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

Answer 46

monoblastic or monocytic

Question 47

What is seen with monoblastic or monocytic AML NOS

Answer 47

Leukemic cells often causes lesions in skin and gums

Question 48

What subtype of AML NOS has Megakaryoblasts cells

Answer 48

Megakaryoblastic

Question 49

What is seen with Megakaryoblastic AML NOS

Answer 49

often associated with significant marrow fibrosis

Question 50

What molecular abnormality has poor prognosis

Answer 50

FTL3 Internal Tandem Duplication (ITD)

Question 51

What 2 molecular abnormalities have a good prognosis

Answer 51

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

Question 52

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

Answer 52

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