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20 Cards in this Set
- Front
- Back
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characteristics of all myeloproliferative DO's.
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originate from pleuripotent hematopoietic progenitor cells. clonal. excessive production of one or more bone marrow elements in the absence of known stimulus. extramedullary hematoppoiesis. prone to acute leukemia.
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tyrosine kinases activated in specefic myeloproliferative DOs?
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chronic myelogenous leukemia is BCR ABL. Polycythemia vera, essential thrombocytosis, and idiopathic myelofibrosis are all Jak II tyr kinase positive.
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what are the proliferating cells in polycythemia vera? chronic myelogenous leukemia? essential thrombocytosis? myelofibrosis?
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erythroid, myeloid, and megakaryocytic elements. myeloid and megakaryocytic elements. megakaryocytic elements. all elements but the fibroblasts become very active in the marrow. note all these diseases can merge into each other
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signs and symptoms of polycythemia vera?
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lots of RBCs, red face, viscous blood, can clot or bleed excessively, headaches, dizziness, systems start to malfunction like GI
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common complication of myeloproliferative disorders as well as lymphoproliferative DOs.
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gout bc high cell turn over leads to lots of purine and pyrimidine synth, uric acid is by product of this.
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if you suspect polycythemia vera, what must you rule out?
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more blood bc there is a physiologic need for it. Like ppl that live in high altitudes, ppl with severe cardiac or respiratory disease. All these issue are known as secondary polycythemia
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age and sex preference of polycythemia vera.
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55 to 65, male over female
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how would you diagnose polycythemia vera?
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two routes. All A signs: total red cell mass over 36ml/kg body mass or 32 for women, arterial O2 saturation over 92%, and splenomegaly. Or B category: first two of A category plus one of the following: thrombocytosis (platelets over 400K), leukocytosis (over 12K), increased leukocyte alanine phosphatase score, serum B12 over 900pg/ml or B12 binding capacity over 2200 pg/ml
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two groups of thought for lab dx of polycythemia.
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Red cell mass vs erythropoietin. EPO group uses decreased EPO, bone marrow, etc. Can also do JAK II assay
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treatments for polycythemia vera.
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phlebotomy to keep Hct under 45% in pts without thrombosis. phlebotomy plus hematological suppression with hydoxyurea in pts without thrombocytosis and previous thrombosis. antiplatelet agents when persistanct thrombocytosis. in eldery pts and those non responsive to the other treatments give radioactive phosphorous
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3 phases of CML?
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chronic phase then accelerated phase and finally blastic phase which is acute leukemia.
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% of specefic myeloproliferative DOs that have constitutively active JAK II
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About 90% of patients with polycythemia vera, are JAK-II positive. 50% of patients with essential thrombocytosis are JAK-II positive. About 40-50% of patients with myelofibrosis are JAK-II positive
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treatments for CML?
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GLEEVEC!! interferon and cytosine arabinoside. hydroxyurea. allogenic bone marrow transplant
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if pt has thrombocytosis, what should your differential include?
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essential thrombocytosis (ie from a bleed and so platelets increase to increase clotting), myelodysplastic syndrome, iron def, inflammatory conditions, splenectomy, occult cancer, polycythemia vera and CML. basically it is a diagnosis of exclusion
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describe the risk stratification in essential thrombocythemia and polycythemia vera (needed for treatment stratification)
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low risk is under 60, no hx of thrombosis, platelet count under 1.5 mil, no CV risk (smoking, hyperlipidemia)... no treatment. high risk is over 60, hx of thrombosis... these guys are treated. intermediate risk is judgement call
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treatment options in essential thrombocytopenia
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over 60 and high risk give hydroxyurea. under 60 and high risk give hydroxyurea or anagleride. women of child bearing age who are high risk give interferon alpha
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main characteristics of myelofibrosis?
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excessive collagen disposition in the bone marrow and extramedullary hematopoiesis
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characteristic cell seen in smear of pt with myelofibrosis.
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tear shaped RBC
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common findings in myelofibrosis
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massive splenomegaly, hepatomegaly, fatigue, anemia, leukocytosis, thrombocytosis
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treatments for myelofibrosis?
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no good ones, chemo, radiation, splenectomy, allopurinol for gout, androgens and steroids for anemia...
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