Malignant Morphology

Question 1

Hematopoietic Marker

Answer 1

CD45

Question 2

Immature Markers

Answer 2

HLA-DR (MHC-II)
CD34
TdT (lymphoid)
CD117 (myeloid)

Question 3

B-cell Markers

Answer 3

CD19
CD20
CD22
CD79a
CD10 (common lymphoid marker)
kappa/lambda light chain
sIg or cIg

Question 4

T-cell Markers

Answer 4

CD1a (immature)
CD2
CD3
CD4
CD5
CD7
CD8
CD99 (immature)

Question 5

NK cell Markers

Answer 5

CD16
CD56

Question 6

Myeloid Markers

Answer 6

CD13
CD14 (monocytic)
CD15
CD33
CD 64/65 (Monocytic)
MPO (lineage defining)
Lysozyme

Question 7

Hyperdiploid B-ALL

Answer 7

51-66 chromosome
DNA index >1.16
Gains of chromosomes 4, 10, 17
Most common cytogenetic abnormality of BALL
Good prognosis

Question 8

Tel-AML

Answer 8

ETV6-RUNX1
t(12;21) - cryptic translocation, needs FISH
Most common translocation in BALL
Good prognosis

Question 9

t(5;14)

Answer 9

IL3-IGH
Profound Eosinophilia
Morphologically distinct BALL

Question 10

t(1;19)

Answer 10

TCF3-PBX1
(E2A-PBX1)

Question 11

DS ALL

Answer 11

Normal cytogenetics
Cryptic CRLF2 translocations but wo poor px

Question 12

MLL ALL

Answer 12

KMT2A
Most common ALL cytogenetics in infants
11q23
BAD prognosis

Question 13

Philadelphia ALL

Answer 13

BCR-ABL 9q22
p190
bad prognosis

Question 14

Ph-like ALL

Answer 14

TKI (EPOR, CRLF2)
IgH
JAK2/3 abberations
IKZF1 deletions - Ikaros (v. bad)
Poor prognosis

Question 15

t(17;19)

Answer 15

TCF3-HLF
hypercalcemia
coagulopathy
bad prognosis

Question 16

Low hypodiploid ALL

Answer 16

32-39 chromosomes
assoc with TP53
very poor prognosis

Question 17

Near haploid ALL

Answer 17

24-31 chromosomes
can masquarade as hyperdiploid, need FISH
results in >4 copies of RUNX1 w/o multiple copies of chromosome 21
bad prognosis

Question 18

Burkitt Leukemia/Lymphoma

Answer 18

deeply basophilic vacuolated cytoplasm
CD45 bright, B-cell markers, surface Ig, negative for TdT and CD34
MYC rearrangements
8;14 MYC;IgH
2;8 MYC; IgK
8;22 MYC;IgL

Question 19

T Lymphoblastic Leukemia/Lymphoma

Answer 19

TCR rearrangements partnered with HOX genes
TAL1 transloacations 1p32
Deletion of 9P
Co-express myeloid markers

Question 20

Early T Precursor Leukemia

Answer 20

CD1a-, CD10-, CD8-, positive for stem cell markers: CD117, CD34, HLA-DR, CD13, CD33, CD11b

Question 21

APML

Answer 21

t(15;17)
PML-RARA
Hypergranular variant
HLA-DR-, CD34-
CD117+, CD64+
Intensely MPO+
Microgranular variant
HLA-DR+, CD34+, CD2+

Question 22

Core Binding Factor AML

Answer 22

Inv16 CBFB-MYH11 FISH
Eosinophilic, dysplasia
t(8;21) RUNX1:RUNX1T1 CBFA
salmon pink granules with long slender auer rods
Defined by cytogenetics not blast count
Good prognosis

Question 23

MLL ALL

Answer 23

KMT2A t(4;11)
Infants
CD10- with myeloid markers

Question 24

MLL AML

Answer 24

MPO- CD14+ CD64+ CD 65+ (monocytic)
Infants often extramedullary chloromas
Many translocations of 11
Biphenotypic and therapy related AML

Question 25

TAM

Answer 25

HLA-DR- CD41+ CD61+ CD4+ CD7+
Big Blue Blebby blasts
GATA1
Resolves <6 mo or evolves to AML
Fibrosis

Question 26

JMML

Answer 26

Hgb F increased, monocytosis
PTPN11 somatic mutation (KRAS, NRAS)
NF1
GMCSF hypersensitivity

Question 27

Hepatosplenic gamma/delta T-cell Lymphoma

Answer 27

Isochromosome 7q
sporadic in teenage males, otherwise immunosuppression is cause

Question 28

HLH

Answer 28

Decreased NK cell function
Assoc with T-cell lymphoma
HSCT for familial

Question 29

GATA2

Answer 29

N371K germline
Multilineage dysplasia

Question 30

Germline predispositions to MDS/AML

Answer 30

Just AML: CEBPA, DDX41
w/plt disorder: RUNX1, ETV6, ANKRD26
Other: GATA2, BMfx, telemoere dysfx, NF1, Noonan, down syndrome

Question 31

CML

Answer 31

Neutrophilia w left shift
Basophilia, eosinophilia
thrombocytosis (acquired VWD)
BCR-ABL p210

Question 32

Polycythemia Vera

Answer 32

JAK2 V617F

Question 33

Essential Thrombocytosis

Answer 33

JAK2 V617F, MPL, CALR