Research Article
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Year 2019, Volume: 6 Issue: 7, 123 - 127, 30.07.2019

Ömer Ekinci Muhammed Aslanboğa

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

Abstract

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.

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

Year 2019, Volume: 6 Issue: 7, 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.
There are 16 citations in total.

Details

Primary Language English
Subjects Health Care Administration
Journal Section Research Article
Authors

Ömer Ekinci 0000-0002-4636-3590

Muhammed Aslanboğa This is me 0000-0001-7856-7005

Publication Date July 30, 2019
Published in Issue Year 2019 Volume: 6 Issue: 7

Cite

APA Ekinci, Ö., & Aslanboğa, M. (2019). Acquired von Willebrand disease in chronic myeloproliferative disorders: a prospective single-center study. Medical Science and Discovery, 6(7), 123-127. https://doi.org/10.17546/msd.584596
AMA Ekinci Ö, Aslanboğa M. Acquired von Willebrand disease in chronic myeloproliferative disorders: a prospective single-center study. Med Sci Discov. July 2019;6(7):123-127. doi:10.17546/msd.584596
Chicago Ekinci, Ömer, and Muhammed Aslanboğa. “Acquired Von Willebrand Disease in Chronic Myeloproliferative Disorders: A Prospective Single-Center Study”. Medical Science and Discovery 6, no. 7 (July 2019): 123-27. https://doi.org/10.17546/msd.584596.
EndNote Ekinci Ö, Aslanboğa M (July 1, 2019) Acquired von Willebrand disease in chronic myeloproliferative disorders: a prospective single-center study. Medical Science and Discovery 6 7 123–127.
IEEE Ö. Ekinci and M. Aslanboğa, “Acquired von Willebrand disease in chronic myeloproliferative disorders: a prospective single-center study”, Med Sci Discov, vol. 6, no. 7, pp. 123–127, 2019, doi: 10.17546/msd.584596.
ISNAD Ekinci, Ömer - Aslanboğa, Muhammed. “Acquired Von Willebrand Disease in Chronic Myeloproliferative Disorders: A Prospective Single-Center Study”. Medical Science and Discovery 6/7 (July 2019), 123-127. https://doi.org/10.17546/msd.584596.
JAMA Ekinci Ö, Aslanboğa M. Acquired von Willebrand disease in chronic myeloproliferative disorders: a prospective single-center study. Med Sci Discov. 2019;6:123–127.
MLA Ekinci, Ömer and Muhammed Aslanboğa. “Acquired Von Willebrand Disease in Chronic Myeloproliferative Disorders: A Prospective Single-Center Study”. Medical Science and Discovery, vol. 6, no. 7, 2019, pp. 123-7, doi:10.17546/msd.584596.
Vancouver Ekinci Ö, Aslanboğa M. Acquired von Willebrand disease in chronic myeloproliferative disorders: a prospective single-center study. Med Sci Discov. 2019;6(7):123-7.
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