Summary
In a review of the literature published in 1984 we identified (from 13 kindreds) 15 patients with severe von Willebrand’s disease (vWD) reported to have developed alloantibodies. In order to reexamine the problem, a questionnaire was sent in 1989 to 141 hemophilia centers in America and Europe; 101 answers were received. These dealt with a total number of 1,720 patients with vWD (all types) and 231 patients with severe (type III) vWD. Besides the 15 cases reported in 1984, 6 additional cases of anti-vWF alloantibodies were reported, i.e., one from Spain (a relative of a previously reported case), two from Venezuela (brother and sister) and three from North Carolina (unrelated patients). All these cases and those previously reported share the following features: occurrence of alloantibodies in multitransfused patients with severe vWD, poor clinical and laboratory- response to replacement therapy with cryoprecipitate, inhibition of ristocetin-induced platelet aggregation. During the survey, an unusual antibody to FVIII:C with no reactivity towards vWF was reported in a multitransfused girl (from Tennessee) with type III vWD. It seems therefore that although the development of alloantibodies is a rare complication of severe vWD, due to its negative effect on the response to treatment it needs a prompt identification.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Bloom A. L., Peake I. R., Furlong R. A., Dames B. L.: High-potency factor VXII concentrate: more effective than cryoprecipitate in a patient with von Willebrand’s disease and inhibitor — Thrombos. Res.16, 847, 1979.
Egberg N., BlombÄck M.: On the characterization of acquired inhibitors to ristocetin-in- duced platelet aggregation found in patients with von Willebrand’s disease — Thrombos. Res.9, 527, 1976.
Hanna W. T.: Replacement therapy for surgery in type III von Willebrand patient with inhibitors to factor VIII procoagulant — Thrombos. Haemostas.62, 224, 1989. (Abstract).
Lenk H., Weissbach G., Donula M.: Ein Hemmkörper bei von Willebrand-Jurgens-Syndrome und seine Wirkung auf die Eigenschaften des Faktor VIII Moleküls — Folia haematol.105, 826, 1978.
Lopez-Fernandez M. F., Martin R., Lopez-Berges C, Ramos F., De Bosch N., Battle J.: Further specific characterization of von Willebrand factor inhibitors developed in two patients with severe von Willebrand disease — Blood72, 116, 1988.
Mannucci P. M., Bloom A. L., Larrieu M. J., Nilsson I. M., West R. R.: Atherosclerosis and von Willebrand factor. I. Prevalence of severe von Willebrand disease in Western Europe and Israel — Brit. J. Haematol.57, 166, 1984.
Mannucci P. M., Mari D.: Antibodies to factor VHI/von Willebrand factor in congenital and acquired von Willebrand’s disease. In:Hoyer L. W. (Ed.): Factor VIII Inhibitors. Alan R. Liss, New York, 1984; p. 109.
Mannucci P. M., Meyer D., Ruggeri Z. M., Koltts J., Clavarella N., Lavergne J. M.: Precipitating antibodies in von Willebrand’s disease — Nature (Lond.)262, 141, 1976.
Mannucci P. M., Ruggeri Z. M., Clavarella N., Kazatchine M. D., Mowbray J. F.: Precipitating antibodies to factor VHI-von Willebrand factor in von Willebrand’s disease: effects on replacement therapy — Blood57, 25, 1981.
Mannucci P. M., Tamaro G., Nerchi G., Candotti G., Federici A. B., Altieri D., Tedesco F.: Life-threatening reaction to factor VIII concentrate in a patient with severe von Willebrand disease and alloantibodies to von Willebrand factor — Europ. J. Hematol.39, 467, 1987.
Maragall S., Castillo R., Ordinas A., Lien-Do F., Rodriguez M.: Inhibition of von Willebrand factor in Willebrand’s disease — Thrombos. Res.14, 495, 1979.
Miller C. H., Bussel J. B., Hilgartner M. W.: Characteristics of inhibitors in severe von Willebrand’s disease — Thrombos. Haemostas.50, 34, 1983.
Ngo K., Glotz V. T., Koziol J. A., Lynch D. C., Gitschier J., Ranieri R. P., Ciavarella N., Ruggeri Z. M., Zimmerman T. S.: Homozygous and heterozygous deletions of the von Willebrand factor gene in patients and carriers of severe von Willebrand’s disease — Proc. nat. Acad. Sci. (Wash.)85, 2753, 1988.
Peake I. R., Liddell C. B., Moodie P., Standen G., Mancuso D. J., Tuley E. A., Westfield L. A., Sorace J. M., Sadler J. E., Verwije C. L., Bloom A. L.: Severe type III von Willebrand’s disease caused by deletion of exon 42 of the von Willebrand factor gene: family studies that identify carriers of the condition and a compound heterozygous individual — Blood75, 654, 1990.
Ruggeri Z. M., Ciavarella N., Bader R., Barbut T: Factor Vlll-related properties in platelets from patients with von Willebrand’s disease — J. Lab. clin. Med.91, 132, 1978.
Ruggeri Z. M., Ciavarella N., Mannucci P. M., Molinari A., Dammacco F., Lavergne J. M., Meyer D.: Familial incidence of precipitating antibodies in von Willebrand’s disease: a study of four cases — J. Lab. clin. Med.94, 60, 1979.
Sarji K. E., Stratton R. D., Wagner R. H., Brinkhous K. M.: Nature of von Willebrand factor. A new assay and a specific inhibitor — Proc. nat. Acad. Sci. (Wash.)71, 2937, 1974.
Shelton-Inloes B. B., Chebab F. F., Mannucci P. M., Federici A. B., Sadler J. E.: Gene deletions correlate with the development of antibodies in von Willebrand disease — J. clin. Invest.79, 1459, 1987.
Shoa’i I., Lavergne J. M., Ajrdaillou N., Obert B., Ala F., Meyer D.: Heterogeneity of von Willebrand’s disease: study of 40 Iranian cases — Brit. J. Haematol.37, 67, 1977.
Stratton R. D., Wagner R. H., Webster W. P., Brinkhous K. M.: Antibody nature of circulating inhibitor of plasma von Willebrand factor — Proc. nat Acad. Sci. (Wash.)72, 4167. 1975.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Mannucci, P.M., Cattaneo, M. Alloantibodies in congenital von Willebrand’s disease. La Ricerca Clin. Lab. 21, 119–125 (1991). https://doi.org/10.1007/BF02919121
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF02919121