AML/ALL

Question 1

AML – Acute myeloid leukemia

Answer 1

leukemic cells resemble cells of one or more myeloid lineages

Question 2

ALL – Acute lymphoblastic leukemia

Answer 2

Leukemic cells - resemble precursor/immature lymphocytes

Question 3

Etiology acute leukemia

Answer 3

Chromosomal abnormality, in PCR - block ability to differentiate/increase autonomy of growth signalling pathway

Question 4

Risk factors for acute leukemia

Answer 4

Chemo (alkylating agent, topoisomerase II), ionizing, tabacco smoke, benzene, genetic syndrome

Question 5

Acute leukemia clinical presentation

Answer 5

Anemia (fatigue, malaise, pallor, dyspnea)
Thrombocytopenia (brusing, petechiae, hemorrhage)
Neutropenia (fever, infection)
Rare: thrombotic events, DIC, skin/gum/tissues

Question 6

___% of cases of ALL occur in children less than ___ y/o

Answer 6

75%; 6 y/o

Question 7

Immaturity markers

Answer 7

CD34

Question 8

Common immature lymphoblasts markers

Answer 8

TdT

Question 9

B cell linage markers

Answer 9

CD19, CD22

Question 10

T cell lineage markers

Answer 10

CD3, CD7

Question 11

B cell mature markers

Answer 11

CD20, surface immunoglobulin

Question 12

B-ALL accounts for _______ % of ALL, typically ALL of ____(age)___

Answer 12

80-85; childhood

Question 13

BCR-ABL1 B-ALL

cytogenetics, distribution, prognosis

Answer 13

t(9;22) - philadelphia chromosome
“Ph+ALL”
25% of adult ALL, 2% childhood ALL
Poor prognosis

Question 14

B-ALL MLL

Answer 14

translocation 11q23
Neonates/young infants
poor prognosis

Question 15

B-ALL ETV6-RUNX1

Answer 15

t(12;21)
25% childhood B-ALL
Favorable prognosis

Question 16

T-ALL traits

Answer 16

25% of ALL
Adolescent/young adults
Mediastinal mass
High WBC
Favors males

Question 17

T-ALL prognosis general trends in children vs adults

Answer 17

Good - kids
>95%, cure80%
Poor adults
Remission 60-80
Cure<50%

Question 18

Poor ALL prognosis factors

Answer 18

< 1 y/o; >10 y/o
Very high WBC
T-lymphoblastic hypodiploidy
Slow response to rx
Min residual disease

Question 19

AML - Acute myeloid leukemia occurs in what age?

Answer 19

adults (65 avg); 10% of childhood leukemia are AML

Question 20

AML diagnosis

Answer 20

> 20% myeloblasts in marrow/peripheral blood

• morphologic appearance
• flow cytometric
• immunohistochemistry

Question 21

Common myeloid markers

Answer 21

CD117 (C-Kit)

Myeloperoxidase

Question 22

AML/APL - PML-RARA/M3 AML

Answer 22

t(15;17)
Blocked at promyeloctyes (not at blasts)
Hypergranular morphology
Auer rods
DIC

Question 23

APL treatment

Answer 23

1. RARA - retinotic acid receptor alpha protein - does not signal well –> accumulation of promyelocyte
   Give high doses of ATRA to overcome

Question 24

AML RUNX1-RUNX1T1

Answer 24

t(8;21)
5% AML - younger patient
Good prognosis

Question 25

APL secondary risk

Answer 25

Disseminated intravascular coagulation (DIC)

Question 26

AML CBFB - MYH11

Answer 26

``` inv(16) or t(16;16) 5-10% AML Younger patients Baso Eos Good prognosis ```

Question 27

AML RBM15 - MKL 1

Answer 27

Megakaryoblastic differenation Infants with downs Good prognosis

Question 28

AML MLL - 11q23

Answer 28

abnormalities of MLL - some monocytic differentiation | Poor prognosis

Question 29

t-AML alkylating agent latency

Answer 29

2-8 years

Question 30

t-AML alkylation agent cytogenetics

Answer 30

whole/partial deletion of 5 and 7

Question 31

t-AML topoisomerase inhibitor

Answer 31

1-2 years | MLL 11q23 rearrangement

Question 32

NOS

Answer 32

Not otherwise specified

Question 33

AML NOS subtype

Answer 33

undifferentiated - myeloblasts Myelomonocytic Monoblasts/monocytic -> skin/gum lesion Megakaryoblastics --> marrow fibrosis

Question 34

AML NOS molecular abnormality

Answer 34

FLT3 - poor NPM1 - good CEBPA - good

Question 35

Hematopoietic stem cell transplant depends on

Answer 35

Younger patient High risk disease Relapse disease Current health standing

Question 36

which t-AML will be de novo?

Answer 36

topoisomerase II

Question 37

Prognosis of t-AML?

Answer 37

very poor

Question 38

Auer Rodes are?

Answer 38

fused azurophilic granules