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excessive numbers of cells in the MYELOID lines myeloproliferative deformed or ineffective cells myelodysplasia Bone marrow fibrosis (scarring) myelofibrosis Secondary myelofibrosis myelophthisis The physician assistant's role is what with myeloproliferative disorders? recognition of overproliferation (refer to hematology) Chronic Myelogenous Leukemia (CML): age, s/s, tx age: 65 s/s: fatigue, anorexia, weight loss, hepatospleenomegaly dx: blood smear (high erythroblasts) Tx: Imatinib (Gleevec) Prognosis: 3-5yrs (chronic-> acute) 3-6m (untreated acute) Polycythemia Vera (age, s/s, tx) age: 60 s/s: pruritis after hot shower (heat activated mast cells), burning feet/lower leg pain, SPLEENomegaly dx: elevated hgb, hct, Leukocyte alkaline phosphate LOW erythropoietin tx: phlebotomy (keep Hct <45), hydroxyurea, and asprin Prognosis: 9-13yrs (treated), 6-18m (untreated) Budd chiari syndrome is associated with which myoproliferative disorder? Polycythemia Vera the purpose of phlebotomy associated with polycythemia vera is to keep the hematocrit below 45 Essential Thrombocytosis has a platelet count greater than 600,000 Essential Thrombocytosis (ET): s/s, dx, tx, prognosis s/s: vasomotor, HA, syncope, chest pain, thrombosis dx: platelet >600,000, No philadelphia chromosone, No reactive thrombocytosis, NO myelofibrosis, Normal iron tx: asprin and refer to hematology (hyrdroxyurea, analgrelide,severe=platelet apharesis) platelet goal of treatment for essential thrombocytosis <500000 Myelofibrosis S/S: bone pain, spleenomegaly, abdominal fullness bruising, pallor, fatigue, SOB due to anemia Dx: bone marrow biopsy Tx: REFER (bone marrow transplant, splenectomy, radiation Prognosis: may live for 10yrs -gradual onset) Which myeloproliferative disease has the most prominent spleenomegaly myelofibrosis The drug associated with defects that is used to treat myelofibrosis Thalidomide (must wear condoms, don't get pregnant, use birth control, ect-- counsel your patient!!) excessive production of myeloid cells is known as CML (Tx: imatinab) excessive production of RBC is known as P. vera (Tx: phlebotomy) excessive production of platelets is known as Essential Thrombocytosis (Tx: asprin and refer) excessive production of megakaryocytes leading to immature cells in peripheral smear Myelofibrosis (Tx: refer, bone marrow transplant, thalidomide)