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Flashcards in Myeloproliferative Disorders Deck (29):
0

6 characteristics of myeloproliferative disorders

-increased number of cells from ONE OR MORE cell lines
-hepatosplenomegally (extramedullary hematopoiesis)
-clonal marrow expansion
-hypercatabolism
-predisposition to AML
-loss of control of proliferation

1

signs of chronic myeloid leukemia

WBC inc
LDH inc
no blasts
inc immature myeloid cells in peripheral blood
hypercellular marrow

2

cytogenics of CML

t (9,22)
diffinitive!

3

what detects Philadelphia chromosome?

florescent in-situ hybridization

4

three phases of CML

chronic phase
accelerated phase
blast phase

5

chronic phase

3 years
high counts, splenomegaly

6

chronic phase treatment

hydroxyurea, start dasatinib

7

accelerated phase

higher and higher counts, splentomegaly
lasts 1 year
more hydroxyurea

8

blast phase

mean survival 3-6 months
similar acute leukemia (>20%)
resistant to treatment- stem cell transplant!

9

FDA and treatment for CML

approves dasatinib, but no evidence that this or nilotinib improve surival

10

average age for polycythemia vera

60

11

unusual symptoms of PV

thombosis
erythomegalia (itchiness)

12

normal in PV

peripheral smear
epo

13

molecular diagnosis

V617F JAK2

14

PV untreated survival

6-9 months

15

target therapy PV

phlebotomy of 1 unit red cells as freq as every 3-4 days with target hematocrit of 40%

16

long term prognosis PV

patients may develop myelofibrosis, secondary AML or MDS; these diseases respond poorly to therapy

17

symptoms essential thrombocytopenia

headache, lightheadedness, syncope, visual changes

18

complications essential thrombocytopenia

thrombosis, abnormal bleeding

19

tests for essential thrombocytopenia

increase number of abnormal megakaryocytes in bone marrow
absence of phil chromosome, reactive thrombocytosis, or iron deficiency

20

ET genetics

V617F JAK2 or W515 L/K

21

therapy for low risk ET

low risk (age <1000)-- no treatment

22

therapy for high risk ET

those not low risk
hydroxyurea (low dose aspirin, anagrelide maybe)
usually can control and wont progress to anything

23

primary myelofibrosis hallmark

splenomegaly

24

other findings in primary myelofibrosis

anemia-->teardrop RBC
increase in abnormal MK in BM
fibrotic BM

25

genetics PM

50% have V617 F JAK2
or W515 L/K

26

prognosis point system

1 for Hgb 30
1 pt for platelet count 1

27

therapy for PM

allogenic stem cell transplant
--others: transfusions for anemia, splenectomy, JAK 2 kinase inhibitors

28

causes of death with PM

bleeding from low platelets
transformation to MDS or AML
infection from low WBC