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Acquired von Willebrand disease.
Mayo Clin Proc 2002; 77(2):181-7MC

Abstract

Acquired von Willebrand disease (AvWD) is a relatively rare acquired bleeding disorder that usually occurs in elderly patients, in whom its recognition may be delayed. Patients usually present predominantly with mucocutaneous bleeding, with no previous history of bleeding abnormalities and no clinically meaningful family history. Various underlying diseases have been associated with AvWD, most commonly hematoproliferative disorders, including monoclonal gammopathies, lymphoproliferative disorders, and myeloproliferative disorders. The pathogenesis of AvWD remains incompletely understood but includes autoantibodies directed against the von Willebrand factor (vWF), leading to a more rapid clearance from the circulation or interference with its function, adsorption of vWF by tumor cells, and nonimmunologic mechanisms of destruction. Laboratory evaluation usually reveals a pattern of prolonged bleeding time and decreased levels of vWF antigen, ristocetin cofactor activity, and factor VIII coagulant activity consistent with a diagnosis of vWD. Acquired vWD is distinguished from the congenital form by age at presentation, absence of a personal and family history of bleeding disorders, and, often, presence of a hematoproliferative or autoimmune disorder. The severity of the bleeding varies considerably among patients. Therapeutic options include desmopressin and certain factor VIII concentrates that also contain vWF. Successful treatment of the associated illness can reverse the clinical and laboratory manifestations. Intravenous immunoglobulins have also shown some efficacy in the management of AvWD, especially cases associated with monoclonal gammopathies. Awareness of AvWD is essential for diagnosis and appropriate management.

Authors+Show Affiliations

Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, Minn 55905, USA.No affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

11838652

Citation

Kumar, Shaji, et al. "Acquired Von Willebrand Disease." Mayo Clinic Proceedings, vol. 77, no. 2, 2002, pp. 181-7.
Kumar S, Pruthi RK, Nichols WL. Acquired von Willebrand disease. Mayo Clin Proc. 2002;77(2):181-7.
Kumar, S., Pruthi, R. K., & Nichols, W. L. (2002). Acquired von Willebrand disease. Mayo Clinic Proceedings, 77(2), pp. 181-7.
Kumar S, Pruthi RK, Nichols WL. Acquired Von Willebrand Disease. Mayo Clin Proc. 2002;77(2):181-7. PubMed PMID: 11838652.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Acquired von Willebrand disease. AU - Kumar,Shaji, AU - Pruthi,Rajiv K, AU - Nichols,William L, PY - 2002/2/13/pubmed PY - 2002/2/28/medline PY - 2002/2/13/entrez SP - 181 EP - 7 JF - Mayo Clinic proceedings JO - Mayo Clin. Proc. VL - 77 IS - 2 N2 - Acquired von Willebrand disease (AvWD) is a relatively rare acquired bleeding disorder that usually occurs in elderly patients, in whom its recognition may be delayed. Patients usually present predominantly with mucocutaneous bleeding, with no previous history of bleeding abnormalities and no clinically meaningful family history. Various underlying diseases have been associated with AvWD, most commonly hematoproliferative disorders, including monoclonal gammopathies, lymphoproliferative disorders, and myeloproliferative disorders. The pathogenesis of AvWD remains incompletely understood but includes autoantibodies directed against the von Willebrand factor (vWF), leading to a more rapid clearance from the circulation or interference with its function, adsorption of vWF by tumor cells, and nonimmunologic mechanisms of destruction. Laboratory evaluation usually reveals a pattern of prolonged bleeding time and decreased levels of vWF antigen, ristocetin cofactor activity, and factor VIII coagulant activity consistent with a diagnosis of vWD. Acquired vWD is distinguished from the congenital form by age at presentation, absence of a personal and family history of bleeding disorders, and, often, presence of a hematoproliferative or autoimmune disorder. The severity of the bleeding varies considerably among patients. Therapeutic options include desmopressin and certain factor VIII concentrates that also contain vWF. Successful treatment of the associated illness can reverse the clinical and laboratory manifestations. Intravenous immunoglobulins have also shown some efficacy in the management of AvWD, especially cases associated with monoclonal gammopathies. Awareness of AvWD is essential for diagnosis and appropriate management. SN - 0025-6196 UR - https://www.unboundmedicine.com/medline/citation/11838652/Acquired_von_Willebrand_disease_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0025-6196(11)62333-1 DB - PRIME DP - Unbound Medicine ER -