Myeloid Issues

Question 1

what can MDS progress to

Answer 1

bone marrow failure

also acute myeloid leukemia

Question 2

what does gains and losses in methylation patterns/genes do

Answer 2

dysregulation of epigenetic programs

Question 3

DNA demethylases (TET’s) do what

Answer 3

DNA demethylation (low levels of methylation)

Question 4

normal bone marrow cellularity %

Answer 4

100-age

Question 5

excessive production of immature forms

Answer 5

acute leukemia

Question 6

excessive production of mature forms

Answer 6

myeloproliferative neoplasm (chronic leukemia)

Question 7

excessive production of dysplastic forms

Answer 7

myelodysplastic syndrome

Question 8

no or very little production of any forms

Answer 8

aplastic anemia

Question 9

MPN can progress to

Answer 9

myelofibrosis, acute leukemia

Question 10

CML defined by

Answer 10

BCR-Abl1 fusion gene on philadelphia chromosome

Question 11

CML presents with

Answer 11

leukocytosis
anemia
splenomegaly

Question 12

t(9;22)

Answer 12

CML: BCR-ABL1 translocation

Question 13

major breakpoint = p210

Answer 13

CML common

Question 14

minor breakpoint = p190

Answer 14

ALL common

Question 15

gleevec targets

Answer 15

increased tyrosine kinase activity of BCR-ABL1 gene

Question 16

blasts in chronic phase CML

Question 17

blasts in CML accelerated phase

Answer 17

10-19%

Question 18

when does CML transform to acute leukemia (blast crisis)

Answer 18

blasts >20%

Question 19

JAK2 mutation

Answer 19

associated w/ almost all polycythaemia vera.
but is nonspecific

50-60% of ET/PMF

Question 20

what causes hemorrhages in P. vera

Answer 20

blood vessel dissension and dysfunctional platelets

Question 21

pathogenesis of primary myelofibrosis

Answer 21

fibrogenetic factors stimulate marrow fibroblast proliferation

Question 22

% of PMF that transforms to acute leukemia

Answer 22

5-30%

Question 23

poikilocytosis with tear-drop (dacrocytes)

Answer 23

PMF

Question 24

osteosclerosis

Answer 24

late-stage PMF

Question 25

ET risks

Answer 25

increased risk for thrombotic and hemorrhagic events

Question 26

ET labs

Answer 26

platelet >450 for 6 months

Question 27

Myelodysplastic syndromes has increased risk of development of

Answer 27

acute myeloid leukemia

Question 28

etiology (therapy related)

Answer 28

exposure to chemotherapeutic agents (alkylating-type) and/or radiation therapy

Question 29

lower risk MDS

Answer 29

RCUD, RARS

Question 30

intermediate risk MDS

Answer 30

RCMD w/ or w/o RS, REAB

Question 31

high risk MDS

Answer 31

RAEB 2

Question 32

good risk categories for MDS

Answer 32

normal cytogenetics
isolated del(5q), (20q), and Y

Question 33

poor risk categories for MDS

Answer 33

complex abnormalities, abnormalities on chromosome 7

Question 34

MDS- nuclear hypolobation

Answer 34

pseudo Pelger-Huet