5

Question 1

AML-M0

Answer 1

minimally differentiated AML

Question 2

AML-M1

Answer 2

AML without maturation

Question 3

AML-M2:

Answer 3

 AML with maturation
 t(8:21): in 20% of cases of AML-M2
 Chloroma: a mass lesion ‘tumor of leukemic cells’

Question 4

AML-M3

Answer 4

 Acute Promyelocytic Leukemia (APL)
 t(15:17): in 100% of cases of AML-M3
 Auer rods and Faggot cells (promyelocytes)
 DIC

Question 5

AML-M4

Answer 5

 Acute myelomonocytic leukemia
 AML-M4E: with eosinophilia
 inv(16) or t(16;16) in AML-M4E (100%)

Question 6

AML-M5

Answer 6

(skin and gum infiltration)
 AML-M5a: Acute monoblastic leukemia
 AML-M5b: Acute monocytic leukemia
 Infiltration of soft tissues (some M4 also)
 Gum infiltration (some M4 also)
 Skin deposits (some M4 also)
 Meningeal involvement-headache, vomiting, eye symptoms (M4)
 AML-M6: Acute erythroleukemia
 AML-M7: Acute megakaryoblastic leukemia
- Prognostic factors
Favorable (good prognosis) Unfavorable

Question 7

AML-M6:

Answer 7

Acute erythroleukemia

Question 8

AML-M7:

Answer 8

Acute megakaryoblastic leukemia

Question 9

Favorable (good prognosis) aml

Answer 9

t (15:17)
t (16:16) = inv(16)
t (8:21)

Question 10

Unfavorable AMD

Answer 10

Age > 60
Prior MDS
Therapy related AML Leukocytosis >100,000/μL Deletion of chromosome 5 or 7

Question 11

t(15;17)

Answer 11

The Promyelocytic leukemia (PML) gene and protein
The retinoic acid receptor (RARA) gene and protein
t(15;17) leads to formation of a hybrid protein
Administration of ATRA (All-Trans Retinoic Acid)

Question 12

The Promyelocytic leukemia (PML) gene and protein

Answer 12

 Located on chromosome 15

 Tumor suppressor gene

Question 13

The retinoic acid receptor (RARA) gene and protein

Answer 13

 Located on chromosome 17
 In presence of ligand (retinoic acid): allows transcription
 In absence of ligand (retinoid acid): prevents transcription

Question 14

t(15;17) leads to formation of a hybrid protein

Answer 14

 No response to physiologic retinoic acid

 Blocks myeloid differentiation at promyelocytic stage

Question 15

Administration of ATRA (All-Trans Retinoic Acid)

Answer 15

 An analogue of vitamin A
 Overcomes the block
 Induces neoplastic promyelocytes to rapidly differentiate into neutrophils

Question 16

AML CD markers

Answer 16

CD11c,CD13,CD14 CD15,CD33,CD64

Question 17

ALL CD markers

Answer 17

CD10,CD19,CD20 CD21,CD23,CD79a

Question 18

B-cell ALL

Answer 18

(85% of cases): Mostly Pre-B cell
 CD19 positive and TdT positive
 Usually manifest in BM and peripheral blood
 Peak at 4 years of age

Question 19

T-cell ALL

Answer 19

25% of cases): Mostly thymic origin
 CD2 positive, CD7 positive, TdT positive
 Mass involving thymus as lymphomas, but can progress rapidly to
leukemic phase, or involve marrow at presentation
 Peak at 15-20 years of age

Question 20

90% of ALLs have nonrandom karyotypic abnormalities true or false

Answer 20

True

Question 21

B- ALL: genes

Answer 21

 t (12;21): ETV6 and RUNX1 genes
 25% of adult pre-B cell tumors: t(9;22) involving ABL and BCR genes  Mutations that inactivate tumors suppressor genes:
 PTEN: mutation → increased pro-growth signaling
 CDKN2A: encodes a negative regulator of the cell cycle and a
positive regulator of p53

Question 22

T –ALL:

Answer 22

diverse chromosomal aberrations

Question 23

Favorable ALL

Answer 23

WBCs < 50,000
Age: 1-10 years 
Female gender
B-cell blasts 
Hyperploidy 
Translocation (12:21) 
Trisomy 4, 10, 17

Question 24

Unfavorable ALL

Answer 24

WBCs  50,000
Age: <1 or  10 years 
Male gender
T-cell blasts
Hypoploidy 
Translocation (9:22) 
Trisomy 5

Question 25

Course ALL

Answer 25

 Rapidly fatal, if untreated

 With chemotherapy, 90% remission rate and 70-80% cure rate

Question 26

Acute Myeloid Leukemia (AML)

Answer 26

• 85% of adult leukemia
• Median age is 50 years
• Most AMLs harbor mutations in genes encoding transcription factors that are
  required for normal myeloid cell differentiation
• Myeloid blasts or promyelocytes make up more than 25% of the bone marrow
  cellular component