Abnormalities of von Willebrand factor in myeloproliferative disease

A relationship with bleeding diathesis

F. Fabris, A. Casonato, M. Grazia Del Ben, L. De Marco, A. Girolami

Research output: Contribution to journalArticle

58 Citations (Scopus)

Abstract

We studied factor VIII related properties in 24 patients with increased platelet numbers. Twenty-one were affected by myeloproliferative disorders (eight had polycythaemia vera, 13 had essential thrombocythaemia) and three had secondary thrombocytosis. Normal levels of VIII:C and VIIIR:Ag were found while a significant (P <0.05) decrease of VIIIR:RCOF (43 ± 13%) related to a lack of larger multimers of VWF (39 ± 12%) was observed in 57% of patients with myeloproliferative disorders. A normal VWF pattern was found in the three patients with secondary thrombocytosis. The highest incidence of VWF abnormalities occurred in patients with essential thrombocythaemia (70%) in comparison with polycythaemic patients (38%). A significant (P <0.03) correlation between platelet count and the values of both VIIIR:RCOF and VWF multimeric pattern was observed only in patients with polycythaemia vera. The lowest levels of VIIIR:RCOF and the greatest loss of larger VWF multimers (less than 30%) were observed in two patients who presented bleeding symptoms at the time of study and a prolonged bleeding time. In addition, the relationship between VWF pattern and bleeding diathesis was supported by the fact that 75% of the patients with VWF abnormalities had bleeding history.

Original languageEnglish
Pages (from-to)75-83
Number of pages9
JournalBritish Journal of Haematology
Volume63
Issue number1
Publication statusPublished - 1986

Fingerprint

Disease Susceptibility
von Willebrand Factor
Hemorrhage
Essential Thrombocythemia
Thrombocytosis
Myeloproliferative Disorders
Polycythemia Vera
Platelet Count
Bleeding Time
Time and Motion Studies
Factor VIII
Incidence

ASJC Scopus subject areas

  • Hematology

Cite this

Fabris, F., Casonato, A., Grazia Del Ben, M., De Marco, L., & Girolami, A. (1986). Abnormalities of von Willebrand factor in myeloproliferative disease: A relationship with bleeding diathesis. British Journal of Haematology, 63(1), 75-83.

Abnormalities of von Willebrand factor in myeloproliferative disease : A relationship with bleeding diathesis. / Fabris, F.; Casonato, A.; Grazia Del Ben, M.; De Marco, L.; Girolami, A.

In: British Journal of Haematology, Vol. 63, No. 1, 1986, p. 75-83.

Research output: Contribution to journalArticle

Fabris, F, Casonato, A, Grazia Del Ben, M, De Marco, L & Girolami, A 1986, 'Abnormalities of von Willebrand factor in myeloproliferative disease: A relationship with bleeding diathesis', British Journal of Haematology, vol. 63, no. 1, pp. 75-83.
Fabris, F. ; Casonato, A. ; Grazia Del Ben, M. ; De Marco, L. ; Girolami, A. / Abnormalities of von Willebrand factor in myeloproliferative disease : A relationship with bleeding diathesis. In: British Journal of Haematology. 1986 ; Vol. 63, No. 1. pp. 75-83.
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