Onkológia S2/2020

Options of treatment for primary myelofibrosis with significant splenomegaly

Introduction: Myelofibrosis (MF) is a Philadelphia-negative myeloproliferative neoplasia characterized by ineffective clonal hematopoiesis, splenomegaly, bone marrow fibrosis, and the risk of progression to acute myeloid leukemia. Symptoms of the disease result form fibrotic remodelling of the bone marrow with subsequent hematopoietic disturbance and the development of extramedullary hematopoiesis in the spleen and liver. The treatment of myelofibrosis depends on the stage of the disease and on the classification in the prognostic risk group. Case: In the article we present the casuistry of a patient with primary myelofibrosis, in whom treatment with ruxolitinib resulted in significant recession of symptoms connected with the disease and reduction of splenomegaly. Conclusion: Our practical experience confirms the benficial clinical effect of treating myelofibrosis with ruxolitinib with an improvement in the patient´s quality of life despite a transient deterioration in hematological parameters for several months.

Keywords: myelofibrosis, splenomegaly, diagnostic criteria, prognostic scoring systems, ruxolitinib