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2014•Acta Haematologica PolonicaRequires access

Pierwotna mielofibroza – przegląd metod leczniczych

Maria Cioch, Paweł Jarosz

Open publisher page 3 citations

Abstract

Primary myelofibrosis (PMF) is a Ph-negative myeloproliferative neoplasm (MPN), that manifests bone marrow fibrosis, cytopenias, splenomegaly, and constitutional symptoms. The conventional therapeutic options for patients with PMF consist of management of anemia, use of cytoreductive and immunomogulatory agents, and splenectomy or splenic irradiation. Cure is only achievable through allogeneic haematopoietic stem cell transplantation. The discovery of crucial role of JAK2 signaling in pathogenesis of PMF has resulting in new JAK2 inhibitor therapy, such ruxolitinib or other investigated molecules.

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What this paper is about

Primary myelofibrosis (PMF) is a Ph-negative myeloproliferative neoplasm (MPN), that manifests bone marrow fibrosis, cytopenias, splenomegaly, and constitutional symptoms. The conventional therapeutic options for patients with PMF consist of management of anemia, use of cytoreductive and immunomogulatory agents, and splenectomy or splenic irradiation. Cure is only achievable through allogeneic haematopoietic stem cell transplantation. The discovery of crucial role of JAK2 signaling in pathogenesis of PMF has resulting in new JAK2 inhibitor therapy, such ruxolitinib or other investigated molecules.

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Available abstract

Primary myelofibrosis (PMF) is a Ph-negative myeloproliferative neoplasm (MPN), that manifests bone marrow fibrosis, cytopenias, splenomegaly, and constitutional symptoms. The conventional therapeutic options for patients with PMF consist of management of anemia, use of cytoreductive and immunomogulatory agents, and splenectomy or splenic irradiation. Cure is only achievable through allogeneic haematopoietic stem cell transplantation. The discovery of crucial role of JAK2 signaling in pathogenesis of PMF has resulting in new JAK2 inhibitor therapy, such ruxolitinib or other investigated molecules.

Key concepts: Myelofibrosis, Ruxolitinib, Myeloproliferative neoplasm, Splenectomy, Medicine, Constitutional symptoms, Extramedullary hematopoiesis, Haematopoiesis

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