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.
Ruxolitinib is the first JAK kinase inhibitor registered for the treatment of primary myelofibrosis and post-polycythaemia vera myelofibrosis and post-essential thrombocytaemia myelofibrosis. The ...article is a summary of current clinical data with ruxolitinib therapy both in myelofibrosis and in other Ph-negative myeloproliferative neoplasms.
pierwotna mielofibroza należy do przewlekłych nowotworów mieloproliferacyjnych o najgorszym rokowaniu, zarówno w odniesieniu do czasu przeżycia, jak i jakości życia. artykuł stanowi podsumowanie ...wiedzy dotyczącej czynników molekularnych i genetycznych związanej z rozwojem choroby, przedstawia także różne wskaźniki prognostyczne i kliniczne oraz możliwości terapii pierwotnej mielofibrozy.
primary myelofibrosis (pmf) belongs to chronic myeloid neoplasms with the worst prognosis in terms of survival and quality of life. This article summarizes current knowledge on molecular and cytogenetic factors related with pmf, presents various stratifications, clinical systems and therapeutic options.
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