Jozef Lukáš, Martin Mistrík
Primary myelofibrosis (PMF) is a rare clonal disorder of a multipotent hematopoietic cell, characterized by extensive marrow fibrosis in the end stage of disease, splenomegaly and extramedullary haematopoiesis. Prognosis of disease depends on risk factors that stratify patients according to Dynamic International Prognostic Scoring System – plus (DIPSS – plus) into 4 groups with low, intermediate – 1, intermediate – 2 and high risk, with a different median survivals – 15,4; 6,5; 2,9 and 1,3 years. Treatment strategy is risk adapted. The only potentially curative treatment is allogeneic hematopoietic stem cell transplantation (HSCT) with a considerable risk of transplant related mortality and chronic morbidity (graft versus host disease). The right ti-ming of allogeneic HSCT is the most difficult and important task in management of PMF disease. Conventional and experimental therapies improve quality of life, but do not improve overall survival.