Acquired von Willebrand Syndrome: Data from an International Registry

Augusto B. Federici; Jacob H. Rand; Paolo Bucciarelli; Ulrich Budde; Perry J. J. van Genderen; Hiroshi Mohri; Dominique Meyer; Francesco Rodeghiero; Evan J. Sadler

doi:10.1055/s-0037-1614018

Thrombosis and Haemostasis, Inhaltsverzeichnis

Thromb Haemost 2000; 84(02): 345-349
DOI: 10.1055/s-0037-1614018

Scientific and Standardization Committee Communication

Schattauer GmbH

Acquired von Willebrand Syndrome: Data from an International Registry

On behalf of the Subcommittee on von Willebrand Factor

Augusto B. Federici

¹From Angelo Bianchi Bonomi Hemophilia Thrombosis Center, Department of Internal Medicine, IRCCS Maggiore Hospital and University of Milan, Italy

,

Jacob H. Rand

²Hematology Division Mount Sinai School of Medicine, New York, USA

,

Paolo Bucciarelli

¹From Angelo Bianchi Bonomi Hemophilia Thrombosis Center, Department of Internal Medicine, IRCCS Maggiore Hospital and University of Milan, Italy

,

Ulrich Budde

³Coagulation Laboratory, Hamburg, Germany

,

Perry J. J. van Genderen

⁴Department of Hematology, University Hospital, Rotterdam, The Netherlands

,

Hiroshi Mohri

⁵Yokohama City University, 1^st Dept of Internal Medicine, Yokohama, Japan

,

Dominique Meyer

⁶Hopital de Bicetre, Paris, France

,

Francesco Rodeghiero

⁷Hemophilia and Thrombosis Center, Department of Hematology, Vicenza, Italy

,

Evan J. Sadler

⁸Howard Hughes Medical Institutions, Washington University, St. Louis, USA

› Institutsangaben

Abstract

Summary

The acquired von Willebrand syndrome (AvWS) is a rare bleeding disorder with laboratory findings similar to those of congenital von Willebrand disease (vWD). Despite the numerous cases reported in the literature until 1999 (n = 266), large studies on AvWS are not available. Moreover, diagnosis of AvWS has been difficult and treatment empirical. These considerations prompted us to organize an international registry. A questionnaire, devised to collect specific information on AvWS, was sent to all the members of the International Society on Thrombosis and Haemostasis (ISTH), who were invited to respond if they had diagnosed cases with the AvWS cases. 156 members answered the questionnaire and 54 of them sent information on 211 AvWS cases from 50 centers. Data were compared with those already published in the literature and 25 cases already described or not correctly diagnosed were excluded. The 186 AvWS cases that qualified for the registry were associated with lymphoproliferative (48%) and myeloproliferative disorders (15%), neoplasia (5%), immunological (2%), cardiovascular (21%) and miscellaneous disorders (9%). Ristocetin cofactor activity (vWF:RCo) or collagen binding activity (vWF:CBA) were usually low in AvWS (median values 20 U/dL, range 3-150), while factor VIII coagulant activity was sometimes normal (median 25 U/dL, range 3-191). FVIII/vWF inhibiting activities were present in only a minority of cases (16%). Bleeding episodes in AvWS were mostly of mucocutaneous type (68%) and were managed by DDAVP (32%), FVIII/vWF concentrates (37%), intravenous immunoglobulins (33%), plasmapheresis (19%), corticosteroids (19%) and immunosuppressive or chemotherapic agents (35%). Based upon the data of this international registry, it appears that AvWS is especially frequent in lympho-or myeloproliferative and cardiovascular diseases. Therefore, AvWS should be suspected and searched with the appropriate laboratory tests especially when excessive bleeding occurs in patients with these disorders. On the basis of the information provided by this registry guidelines for diagnosis and management of the AvWS are given.

Keywords

Acquired von Willebrand syndrome - diagnosis - therapy

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Referenzen

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