Myeloproliferative Disorders Flashcards
signs of chronic myeloid leukemia
WBC inc LDH inc no blasts inc immature myeloid cells in peripheral blood hypercellular marrow
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
cytogenics of CML
t (9,22)
diffinitive!
what detects Philadelphia chromosome?
florescent in-situ hybridization
three phases of CML
chronic phase
accelerated phase
blast phase
chronic phase
3 years
high counts, splenomegaly
chronic phase treatment
hydroxyurea, start dasatinib
accelerated phase
higher and higher counts, splentomegaly
lasts 1 year
more hydroxyurea
blast phase
mean survival 3-6 months
similar acute leukemia (>20%)
resistant to treatment- stem cell transplant!
FDA and treatment for CML
approves dasatinib, but no evidence that this or nilotinib improve surival
average age for polycythemia vera
60
unusual symptoms of PV
thombosis
erythomegalia (itchiness)
normal in PV
peripheral smear
epo
molecular diagnosis
V617F JAK2
PV untreated survival
6-9 months
target therapy PV
phlebotomy of 1 unit red cells as freq as every 3-4 days with target hematocrit of 40%
long term prognosis PV
patients may develop myelofibrosis, secondary AML or MDS; these diseases respond poorly to therapy
symptoms essential thrombocytopenia
headache, lightheadedness, syncope, visual changes
complications essential thrombocytopenia
thrombosis, abnormal bleeding
tests for essential thrombocytopenia
increase number of abnormal megakaryocytes in bone marrow
absence of phil chromosome, reactive thrombocytosis, or iron deficiency
ET genetics
V617F JAK2 or W515 L/K
therapy for low risk ET
low risk (age <1000)– no treatment
therapy for high risk ET
those not low risk
hydroxyurea (low dose aspirin, anagrelide maybe)
usually can control and wont progress to anything
primary myelofibrosis hallmark
splenomegaly
other findings in primary myelofibrosis
anemia–>teardrop RBC
increase in abnormal MK in BM
fibrotic BM
genetics PM
50% have V617 F JAK2
or W515 L/K
prognosis point system
1 for Hgb 30
1 pt for platelet count 1
therapy for PM
allogenic stem cell transplant
–others: transfusions for anemia, splenectomy, JAK 2 kinase inhibitors
causes of death with PM
bleeding from low platelets
transformation to MDS or AML
infection from low WBC
