Bone Marrow malignacies

Question 1

CD34+

Answer 1

blasts

Question 2

CD34+, CD33+

Answer 2

myeloid blasts

Question 3

Tdt+, CD10+

Answer 3

lymphoid blasts

Question 4

CD19+, CD20+

Answer 4

mature B-lymphocytes/lymphomas

Question 5

CD3+, CD5+

Answer 5

Mature T-lymphocytes/lymphmoas

Question 6

T(8;21)

Answer 6

RUNX1-RUNX1T1
AML subtype seen in kids; CD34+, HLA-DR+, CD13+
weak CD33
treat with chemo

Question 7

inv (16) (p13.1; q22) or t(16:16)(p13.1;q22)

Answer 7

CBFB-MYH11
AML subtype, seen in kids; mixed granulocyte-monocyte features; increased eosinophils
positive for CD34, CD117, CD13, CD33, CD14, CD11b
treat with particular chemo

Question 8

T(15;17)(q22:q12)

Answer 8

PML-RARA
AML subtype; severe thrombocytopenia; blasts with “bat-wing” nuclei, Auer rods; absent CD34, HLA-DR
CD13+, CD33+
treated with ATRA

Question 9

AML with complex karyotype

Answer 9

any age; poor prognosis; p53 mutation

CD34+, CD117 and CD33+

Question 10

T(9:22) (q34;q11.2) p190

Answer 10

BCR-ABL1; proliferation activator; often with maturation inhibiting mutation 
B-ALL; older cases
big agranular blasts
CD10+, CD19+, TdT+
bad prognosis

Question 11

T(v;11q23)

Answer 11

MLL rearranged--maturation inhibitor 
B-ALL; in babies
big agranular blasts
CD10-
CD19+, TdT+
bad prognosis

Question 12

T(12:21)

Answer 12

TEL-AML1--prevents maturation
B-ALL; 25% of affected kids
big agranular blasts
TdT+, CD34+, CD10+ CD20-
good prognosis

Question 13

Hyperdiploid (>50 chromosomes)

Answer 13

B-ALL

good prognosis

Question 14

T-ALL

Answer 14

translocation of oncogene and TCR; 
kids; often with thymic masses or lymph involvement
big agranular blasts
TdT+, CD3+, CD5+
high risk

Question 15

t(9:22)(q34;q11.2)

p210

Answer 15

CML treat with Imitimab

Question 16

Polycythemia Vera

Answer 16

myeloprlifreative disease with increased RBCs and Megs; activation of Jak2 (no increased EPO);
presents with thrombosis
can progress to myelofibrosis, MDS, ALL

Question 17

Essential thrombocythemia

Answer 17

increased platelets (rarer than Fe def), possible Jak2 mutation; increased Megs (large and weird looking, and cluster together)
can progress to other diseases

Question 18

Primary Myelofibrosis

Answer 18

poorly understood; some Jak2 mutations; presents with thrombus; Increased Megs (bizarre shapes, clustering) fibrosis; can progress to marrow failure, acute leukemia

Question 19

Mastocytosis

Answer 19

increased mast cells; presents w/itchy, pigmented skin lesion; typically don’t spread but will go to bone marrow>lymph nodes, spleen, liver
aggregates of bland looking cells, eosinophilia
cKIT mutation or PDGFRA activation
CD117, CD25, tryptase positive

Question 20

MDS w/ isolated del(5q)

Answer 20

Meg are mononuclear; presents-old people with severe anemia, women;
treated with lenalidomide
10% progress to AML