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Background: The treatment of patients with polycythemia vera (PV) and essential thrombocythemia (ET) is conducted according to well-defined risk stratification systems. We hypothesized that adherence to the guidelines, namely the decision to refrain from introducing cytoreduction in non-high-risk patients, is particularly difficult in patients diagnosed when they are between 40 and 59 years of age (intermediate-age group)., Objectives: To evaluate the group of intermediate-age PV and ET patients, focusing on a first-line treatment approach adapted at diagnosis., Material and Methods: The study group consisted of 308 PV and ET patients recruited from 6 Polish Adult Leukemia Group (PALG) Centers. Patients were analyzed with respect to disease phenotype, risk group, treatment approach, cardiovascular (CV) risk factors, and occurrence of bleeding or thrombosis., Results: Overall, 74% of patients in the study group were started on cytoreduction at diagnosis, including 70% of the low-risk PV patients and 85-89% of the non-high-risk ET patients. Factors influencing the decision to start the treatment included higher hemoglobin (Hb) concentration (in PV) as well as higher platelet (PLT) count, and the presence of CV risk factors (in ET). Introducing cytoreduction at diagnosis had no impact on thrombotic events. Patients harboring CV risk factors experienced a higher incidence of complications both at diagnosis and follow-up, independently of the treatment strategy., Conclusions: We underline the low adherence to recommendations in the treatment of intermediate-age PV and ET patients. Moreover, we emphasize the importance of CV risk factors and stress their impact on disease phenotype in this patient population.

Background: Identification of patients with myelofibrosis being at increased risk of acquired von Willebrand syndrome (avWS) would likely facilitate individualization of treatment and improve its outcomes., Objectives: To determine the prevalence of avWS in patients with myelofibrosis, and to verify if individuals with and without this bleeding disorder differ in terms of their baseline clinical parameters., Material and Methods: The study included 32 consecutive patients with myelofibrosis. avWS was diagnosed on the basis of abnormally low levels of von Willebrand factor and other routine tests. Patients with and without concomitant avWS were compared in terms of their demographic characteristics, present and past medical histories and laboratory parameters., Results: Concomitant avWS was found in 5 patients (15.6%). In 1/5 patients with avWS and in 8/27 persons without this bleeding disorder, myelofibrosis developed secondarily to polycythemia vera (n = 7) or essential thrombocytopenia (n = 2). As many as 4/5 individuals with avWS presented with clinical evidence of a bleeding disorder. The subjects with avWS differed from the remaining patients with myelofibrosis in terms of significantly lower activity of von Willebrand factor (vWF) and lower vWF to vWF antigen ratio., Conclusions: All patients with myelofibrosis should be routinely evaluated for avWS with the panel of specific tests. Further, avWS should be the primary suspicion in each patient with myelofibrosis in whom clinical evidence of a bleeding disorder has emerged.