mpnmds Flashcards
what is the defeinition of a myeloproliferative neoplasms
group of clonol myeloid neoplasma characterized by proliferation of one or more cell type in peripheral blood with minimal dysplasia.
what are some common feature of myeloproliferative neoplasms
basophilia and splenomegaly
what is the median age of CML diagnosis
46-53 years of age
what percent of people with CML are asymptomatic
40.00%
what are the pathological features of the chronic phase of CML
peripheral blood left shifted more granulocytosis in all states netrophilia with myelocyte bulge.
what do you see in the chronic phase of CML in the Bone marrow aspirate or biopsy
markedly hyper-cellular
what is the difference between the major minor and kilodaltion breakpoints for BCR ABL of CML
major p210 vast majority, minor p190, kilodaltion p230 cml with thrombosis
what is CML in the accelerated phase
peripheral blood or BM blasts in less than 20% PB basophilia less than 20%
what is CML in the blast phase
PB or BM greater than 20%
tell me about CML in general
tyrosine kinase overactivity due to a translocation of chormosones 9 and 22
what is the treatment of CML
imatanib or stem cell tranplantation for younger patients
what is the prognosis of CML
4-6 years in the chornic phase then in the terminal blast phase with imatinib prolonged survival.
what do you see in leukemoid reactions
Nml resonse to infection segs and bands no basophilia or splenomegaly LAP score elevated normal megas
what do you see in the CML
clonol MPN myelocyte bulge basophilia and splenomegaly LAP score decreased dwarf megas
what is polycythemia vera
increased RBC mass RBC HGB and HCT, medain age about 60
what are the clinical features of polycythemia VERA PV
hyperviscosity related symptoms pruritus after warm shower.
what is the diagnosis criteria for PV
2 major and 1 minor or I major and 2 minor, major criteria HGB >18.5 men 16.5 women JAK2 mutation, minor hypercellular marrow with panmyelosis low serum epo endogenous erythroid colony formation in vitro.
what are the pathological features of polycythemic phase
peripheral blood erythrocytosis and in the bone marrow hyperceulluar with panmyelosis erythroid predominance variable enlarged megakaryocytes and clustered.
what are the pathological features of the spent phase of PV
leukoerytrhoblastosis left shifrted neutrophils and tear drob shaped RBC bone marrow often a dry tap increasing fibrosis
what is the pathophysiology of PV
JAK2 tyrosine kinase point mutation accounts for more than 95%
what is the treatment of PV
blood letting myelosuppresive drugs 15 year survival of 65% worse with history of thrombosis
what are some differnentail diagnosis of r PV
secondary polycythemina due to smoking high altitude ECT>
how can you differnatial between secondary polycythemia and PV
clonol MPN decreased EPO splenomegaly penlarged megas in PV
what is Essential thrombocytosis
increased platelat production more than 450k
