Acquired von Willebrand disease in chronic myeloproliferative disorders: a prospective single-center study

Abstract

Objective:  The rate of acquired von Willebrand disease (aVWD) among myeloproliferative patients is substantial enough to merit serious consideration, as it is thought to play a role in hemorrhage. We aimed to investigate the rate of acquired von Willebrand disease (aVWD) in chronic myeloproliferative disorders (MPD) Materials and Methods: The present study was conducted prospectively on 70 patients admitted to hematology clinic. Complete blood count, PT, aPTT, vWF:Ag level, vWF:RCoF test, and factor VIII levels were analyzed for all patients. A finding of vWF:RCoF / Ag < 0.7 was accepted as the predisposition to aVWD. Results: Of the patients, 33 (47.1%) were male, 37 (52.8%) were female, and the mean age was 50 ± 16.25. We detected aVWD in 19 (vWF:RCoF / Ag test < 0.7) (28%) of the 70 patients in the study group. Predisposition to aVWF was present in 7 of the 16 patients in the ET group(43.7%), in 4 of the 11 PV patients (36%), and in 8 of the 43 CML patients (18.6%). There was no statistically significant difference in the presence of aVWD between the three disease groups (p: 0.079). Conclusion: The underlying mechanism of aVWD is still not fully resolved. Myeloproliferative diseases are one of the few diseases that can cause avWS. It should be kept in mind that aVWD may play a role in pathogenesis in people with chronic myeloproliferative disease, especially in cases of hemorrhage occurring in ET and PV patients.

Keywords

• Acquired von Willebrand disease,chronic myeloproliferative disorders

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