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SBM > Myeloproliferative Disorders > Flashcards

Myeloproliferative Disorders Flashcards

0
Q

signs of chronic myeloid leukemia

A 
WBC inc
LDH inc
no blasts
inc immature myeloid cells in peripheral blood
hypercellular marrow
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1
Q

6 characteristics of myeloproliferative disorders

A
  • 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
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2
Q

cytogenics of CML

A

t (9,22)

diffinitive!

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3
Q

what detects Philadelphia chromosome?

A

florescent in-situ hybridization

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4
Q

three phases of CML

A

chronic phase
accelerated phase
blast phase

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5
Q

chronic phase

A

3 years

high counts, splenomegaly

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6
Q

chronic phase treatment

A

hydroxyurea, start dasatinib

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7
Q

accelerated phase

A

higher and higher counts, splentomegaly
lasts 1 year
more hydroxyurea

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8
Q

blast phase

A

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

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9
Q

FDA and treatment for CML

A

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

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10
Q

average age for polycythemia vera

A

60

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11
Q

unusual symptoms of PV

A

thombosis

erythomegalia (itchiness)

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12
Q

normal in PV

A

peripheral smear

epo

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13
Q

molecular diagnosis

A

V617F JAK2

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14
Q

PV untreated survival

A

6-9 months

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15
Q

target therapy PV

A

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

16
Q

long term prognosis PV

A

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

17
Q

symptoms essential thrombocytopenia

A

headache, lightheadedness, syncope, visual changes

18
Q

complications essential thrombocytopenia

A

thrombosis, abnormal bleeding

19
Q

tests for essential thrombocytopenia

A

increase number of abnormal megakaryocytes in bone marrow

absence of phil chromosome, reactive thrombocytosis, or iron deficiency

20
Q

ET genetics

A

V617F JAK2 or W515 L/K

21
Q

therapy for low risk ET

A

low risk (age <1000)– no treatment

22
Q

therapy for high risk ET

A

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

23
Q

primary myelofibrosis hallmark

A

splenomegaly

24
Q

other findings in primary myelofibrosis

A

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

25
Q

genetics PM

A

50% have V617 F JAK2

or W515 L/K

26
Q

prognosis point system

A

1 for Hgb 30

1 pt for platelet count 1

27
Q

therapy for PM

A

allogenic stem cell transplant

–others: transfusions for anemia, splenectomy, JAK 2 kinase inhibitors

28
Q

causes of death with PM

A

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

SBM (18 decks)

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