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Acquired von Willebrand syndrome: data from an international registry.
Thromb Haemost 2000; 84(2):345-9TH

Abstract

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.

Authors+Show Affiliations

Angelo Bianchi Bonomi Hemophilia Thrombosis Center, Department of Internal Medicine, IRCCS Maggiore Hospital and University of Milan, Italy. Augusto.Federici@unimi.itNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Guideline
Journal Article
Practice Guideline

Language

eng

PubMed ID

10959711

Citation

Federici, A B., et al. "Acquired Von Willebrand Syndrome: Data From an International Registry." Thrombosis and Haemostasis, vol. 84, no. 2, 2000, pp. 345-9.
Federici AB, Rand JH, Bucciarelli P, et al. Acquired von Willebrand syndrome: data from an international registry. Thromb Haemost. 2000;84(2):345-9.
Federici, A. B., Rand, J. H., Bucciarelli, P., Budde, U., van Genderen, P. J., Mohri, H., ... Sadler, J. E. (2000). Acquired von Willebrand syndrome: data from an international registry. Thrombosis and Haemostasis, 84(2), pp. 345-9.
Federici AB, et al. Acquired Von Willebrand Syndrome: Data From an International Registry. Thromb Haemost. 2000;84(2):345-9. PubMed PMID: 10959711.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Acquired von Willebrand syndrome: data from an international registry. AU - Federici,A B, AU - Rand,J H, AU - Bucciarelli,P, AU - Budde,U, AU - van Genderen,P J, AU - Mohri,H, AU - Meyer,D, AU - Rodeghiero,F, AU - Sadler,J E, AU - ,, PY - 2000/8/26/pubmed PY - 2001/2/28/medline PY - 2000/8/26/entrez SP - 345 EP - 9 JF - Thrombosis and haemostasis JO - Thromb. Haemost. VL - 84 IS - 2 N2 - 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. SN - 0340-6245 UR - https://www.unboundmedicine.com/medline/citation/10959711/Acquired_von_Willebrand_syndrome:_data_from_an_international_registry_ L2 - http://www.diseaseinfosearch.org/result/9536 DB - PRIME DP - Unbound Medicine ER -