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Acquired von Willebrand disease.
Baillieres Clin Haematol 1998; 11(2):319-30BC

Abstract

Acquired von Willebrand disease (AvWD) is an acquired bleeding disorder which may suddenly become manifest in individuals, usually in the absence of a personal or family history of bleedings and frequently in association with monoclonal gammopathies, lymphoproliferative, myeloproliferative and autoimmune disorders. In a minority of the cases AvWD may develop in association with drugs or solid tumours. Pathogenetic mechanisms involve autoantibodies directed against von Willebrand factor (vWF) resulting in a rapid clearance of vWF from the circulation and/or inactivation of plasma vWF; absorption or adsorption of plasma vWF to malignant cells; drug-induced or cell-mediated proteolysis of plasma vWF; acquired decrease in synthesis of vWF and/or release of vWF from storage sites; or precipitation of plasma vWF. Treatment options include--whenever possible--treatment of the underlying disorder or symptomatic treatment aimed at replacing the loss of vWF by either infusion of vWF-rich concentrates or administration of desmopressin (DDAVP). In selected cases with anti-vWF antibodies, administration of high-dose intravenous gammaglobulin, plasma exchange or extracorporeal immunoadsorption may be successful.

Authors+Show Affiliations

Department of Haematology, University Hospital Dijkzigt, Rotterdam, The Netherlands.No affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

10097810

Citation

van Genderen, P J., and J J. Michiels. "Acquired Von Willebrand Disease." Bailliere's Clinical Haematology, vol. 11, no. 2, 1998, pp. 319-30.
van Genderen PJ, Michiels JJ. Acquired von Willebrand disease. Baillieres Clin Haematol. 1998;11(2):319-30.
van Genderen, P. J., & Michiels, J. J. (1998). Acquired von Willebrand disease. Bailliere's Clinical Haematology, 11(2), pp. 319-30.
van Genderen PJ, Michiels JJ. Acquired Von Willebrand Disease. Baillieres Clin Haematol. 1998;11(2):319-30. PubMed PMID: 10097810.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Acquired von Willebrand disease. AU - van Genderen,P J, AU - Michiels,J J, PY - 1999/3/31/pubmed PY - 1999/3/31/medline PY - 1999/3/31/entrez SP - 319 EP - 30 JF - Bailliere's clinical haematology JO - Baillieres Clin. Haematol. VL - 11 IS - 2 N2 - Acquired von Willebrand disease (AvWD) is an acquired bleeding disorder which may suddenly become manifest in individuals, usually in the absence of a personal or family history of bleedings and frequently in association with monoclonal gammopathies, lymphoproliferative, myeloproliferative and autoimmune disorders. In a minority of the cases AvWD may develop in association with drugs or solid tumours. Pathogenetic mechanisms involve autoantibodies directed against von Willebrand factor (vWF) resulting in a rapid clearance of vWF from the circulation and/or inactivation of plasma vWF; absorption or adsorption of plasma vWF to malignant cells; drug-induced or cell-mediated proteolysis of plasma vWF; acquired decrease in synthesis of vWF and/or release of vWF from storage sites; or precipitation of plasma vWF. Treatment options include--whenever possible--treatment of the underlying disorder or symptomatic treatment aimed at replacing the loss of vWF by either infusion of vWF-rich concentrates or administration of desmopressin (DDAVP). In selected cases with anti-vWF antibodies, administration of high-dose intravenous gammaglobulin, plasma exchange or extracorporeal immunoadsorption may be successful. SN - 0950-3536 UR - https://www.unboundmedicine.com/medline/citation/10097810/Acquired_von_Willebrand_disease_ L2 - http://www.diseaseinfosearch.org/result/7418 DB - PRIME DP - Unbound Medicine ER -