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

Year 2019, , 123 - 127, 30.07.2019

Ömer Ekinci , Muhammed Aslanboğa

https://doi.org/10.17546/msd.584596

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

References

• 1. Federici AB, Rand JH, Bucciarelli P, Budde U, van Genderen PJ, Mohri H, Meyer D, Rodeghiero F, Sadler JE; Subcommittee on von Willebrand Factor. Acquired von Willebrand syndrome: data from an international registry. Thromb Haemost. 2000;84(2):345-349.
• 2. Van Genderen PJ, Michiels JJ. Acquired von Willebrand disease. Baillieres Clin Haematol. 1998;11(2):319-330.
• 3. Mital A, Prejzner W, Swiatkowska-Stodulska R, Hellmann A. Factors predisposing to acquired von Willebrand syndrome during the course of polycythemia vera – retrospective analysis of 142 consecutive cases. Thromb Res. 2015;136(4):754-757.
• 4. Mital A, Prejzner W, Bieniaszewska M, Hellmann A. Prevalence of acquired von Willebrand syndrome during essential thrombocythemia: a retrospective analysis of 170 consecutive patients. Pol Arch Med Wewn. 2015;125(12):914-920.
• 5. Tiede A, Priesack J, Werwitzke S, et al. Diagnostic workup of patients with acquired von Willebrand syndrome: a retrospective single-centre cohort study. J Thromb Haemost. 2008;6(4):569-576.
• 6. Federici AB. Acquired von Willebrand syndrome: an underdiagnosed and misdiagnosed bleeding complication in patients with lymphoproliferative and myeloproliferative disorders. Semin Hematol. 2006;43(1):48-58.
• 7. De Meyer SF, Deckmyn H, Vanhoorelbeke K. von Willebrand factor to the rescue. Blood. 2009;113(21):5049-5057.
• 8. Goldman JM, Melo JV. Chronic myeloid leukemia–advances in biology and new approaches to treatment. N Engl J Med. 2003 v. 349(15):1451-1464.
• 9. Tiede A, Rand JH, Budde U, Ganser A, Federici AB. How I treat the acquired von Willebrand syndrome. Blood. 201;117(25):6777-6785.
• 10. Castaman G, Lattuada A, Ruggeri M, et al. Platelet von Willebrand factor abnormalities in myeloproliferative syndromes. Am J Hematol. 1995;49(4):289-293.
• 11. Franchini M, Lippi G. Acquired von Willebrand syndrome: an update. Am J Hematol. 2007; 82 (5): 368–375.
• 12. Fabris F, Casonato A, Del Ben MG, et al. Abnormalities of von Willebrand factor in myeloproliferative disease: a relationship with bleeding diathesis. Br J Haematol. 1996;63(1):75-83.
• 13. Tatewaki W, Takahashi H, Shibata A, et al. Multimeric composition of plasma von Willebrand factor in chronic myelocytic leukaemia.Thromb Res. 1988;52(1):23-32.
• 14. Rupa-Matysek J, Lewandowski K, Lewandowska M, et al. Bleeding complications after arthroscopy in a JAK2V617F-positive patient with essential thrombocythemia and acquired von Willebrand syndrome (AVWD). Int J Hematol. 2015;101(4):405-410
• 15. Budde U, Schaefer G, Mueller N, et al. Acquired von Willebrand’s disease in the myeloproliferative syndrome. Blood. 1994;64(5):981-985.
• 16. Levy GG, Nichols WC, Lian EC, et al. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura Nature. 2001 Oct 4;413(6855):488-494.