Myeloproliferative Disorders

Gleevec for Polycythemia Vera – pro

Gleevec is a drug initially approved for Chronic Myelogenous Leukemia. It wasrecently  studied for P. Vera by Richard Silver at Weil-Cornell and the study was published in 2011. It described 37 patientswith an overall response of 49%. Thirty percent had a complete response, and 19%, a partial response. However, a previous study by Nussenzveig et al, did not show much activity in 27 patients. Overall, 4 (17%) patients responded: one had a complete

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Allogeneic SCT for Myelofibrosis – pro

Myelofibrosis with myeloid metaplasia (MMM) is a chronic myeloproliferative disorder characterized by bone marrow fibrosis and extramedullary hematopoiesis. Recently, a retrospective analysis of the outcomes of 320 patients with CIMF receiving allo-SCT between 1989 and 2002 was published. Most patients received ablative conditioning with either total body irradiation (TBI) (n = 117) or busulphan (n = 150) and cyclophosphamide. Bone marrow was the

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JAK2 for diagnosis – pro

Lay Summary: JAK2 testing can now be performed for a diagnosis of a myeloproliferative disorder.  This is now an acceptable approach to diagnosing myeloproliferative disorders. In early 2005, several groups of investigators reported a somatic acquired point mutation in the JAK2 (Janus kinase 2) protein in the blood and bone marrow of patients with BCR/ABL-negative chronic myeloproliferative disorders. JAK2 is a tyrosine kinase which plays an important

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Pegasys for Myelofibrosis – pro

Interferon is effective in myeloproliferative disorders, which include myelofibrosis. Pegylated interferon is more convenient that interferon because it can be given weekly. However, in the one study in which marrow histology was examined, progression of myelofibrosis was not inhibited by pegylated interferon. Many patients with myelofibrosis are JAK2 positive and pegylated interferon has been shown to reduce Jak2 expression. However, the extent

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Juvenile Myelomonocytic Leukemia – pro

Smith FO, Loh ML. Myelodysplastic and myeloproliferative syndrome in children. In: Hoffman R, Benz EJ, Shattil SJ, Furie B, Silberstein LE, McGlave P, et al., editors. Hematology: basic principles and practice. 5th ed. Philadelphia (PA): Churchill Livingstone; 2008. Juvenile myelomonocytic leukemia is a type of a myelodysplastic syndrome. The only treatment that has resulted in cures for JMML is a bone marrow transplant, with about a 50% survival

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Erytropoietin therapy for myelofibrosis – pro

Experience with recombinant human erythropoietin (rHuEPO) in the treatment of the anemia secondary to myelofibrosis with myeloid metaplasia (MMM) is limited.There are a number of reports of small numbers of patiens demonstrating responsiveness but also a recent report  which presents a picture of non-responsiveness of transfusion dependent myelofibrosis to erythropoietin therapy. Initial studies failed to show significant improvements in hemoglobin

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Pegasys for P. Vera and Essential Thrombosis – pro

Intron A is a well established treatment for essential thrombocythemia (ET) and P. Vera, especially effective for control of pruritis. Interferon-(IFN) suppresses growth of multipotent hematopoietic progenitor cells. No leukemogenic risk has been reported. No randomized trials have been published. In the Italian guidelines for treatment of ET 15 studies have been summarized, including 292 patients. Complete normalization of platelet counts was achieved

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Interferon for hypereosinophilic syndrome – pro

Chronic myeloproliferative disorders (CMPD) are classified according to the WHO classification of 2001 as polycythemia vera (PV), chronic idiopathic myelofibrosis (CIMF), essential thrombocythemia (ET), CMPD/unclassifiable (CMPD-U), chronic neutrophilic leukemia, and chronic eosinophilic leukemia (CEL)/hypereosinophilic syndrome, all to be delineated from BCR/ABL-positive chronic myeloid leukemia (CML). Hypereosinophilic syndromes (HES) and systemic

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