(診斷) 骨髓纖維化 Primary myelofibrosis can begin with a blood picture suggestive of Polycythemia Vera or CML/ Most patients have moderate to severe anemia/ Eventually the patient develops thrombocytopenia/ The peripheral smear appears markedly abnormal/ Red cell abnormality includes bizarre shapes (teardrop-shaped RBCs on a peripheral smear). Nucleated erythroid precursors are seen in the peripheral blood/ Immature white cells are also seen and basophils are increased /Dry tap on bone marrow aspiration/ Reticulin or collagen fibrosis in bone marrow biopsy.
諾華ruxolitinib (Rx) - Jakafi for Myelofibrosis Kinase inhibitor indicated for treatment of patients with intermediate or high-risk myelofibrosis, including primary myelofibrosis, post-polycythemia vera myelofibrosis and post-essential thrombocythemia myelofibrosis
Initial dose (platelet count >200 x10^9/L): 20 mg PO q12hr
Initial dose (platelet count 100-200 x10^9/L): 15 mg PO q12hr
Initial dose (platelet count 50 to <100 x10^9/L): 5 mg PO q12hr
Titrate dose based on response; not to exceed 25 mg PO q12hr
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