Screening of acqui̇red von willebrand disease and frequency of bleeding in BCR-ABL negative chronic myeloproliferative diseases

Objective: It was aimed to determine the acquired Von Willebrand disease (AVWS) and bleeding frequency in patients with Bcr-abl negative CMPN. It was also aimed to investigate the determining risk factors for bleeding and AVWS development. Material and Method: In this cross-sectional study, a total of 50 patients including 20 ET, 20 PV, 10 PMF, who applied to the adult hematology clinic of Manisa Celal Bayar University Medical Faculty and whose diagnoses were revised according to the last updated WHO 2016 criteria were included. Patients were tested for complete blood count, peripheral smear, blood group, prothrombin time (PT), activated partial thromboplastin time (APTT), fibrinogen, Factor VIII, VWF antigen level (VWF:Ag), Ristocetin cofactor activity (Rcof) and bleeding time tests. Results: AVWS developed in 55% (n=11) of the ET group, 20% (n=4) of the PV group and 20% (n=2) of the PMF group. AVWS was most frequently detected in the ET group. Platelet levels of patients with AVWS were found statistically significantly higher than patients without AVWS. Bleeding was observed in 48% of all patients. Both bleeding and thrombosis development rates were significantly higher in patients with splenomegaly during diagnosis. Conclusion: In our study, platelet levels of patients with AVWS were found to be statistically significantly higher than patients without AVWS. Large-scale and multicentre studies involving more patients are needed to determine the frequency of AVWS and to reveal risk groups in bcr-abl negative chronic myeloproliferative neoplasms. Key Words: Acquired Von Willebrand Disease (AVWS), Bleeding, Chronic Myeloproliferative Neoplasms