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Use of intravenous immunoglobulin in patients with acquired von Willebrand syndrome.
Hum Immunol. 2005 Apr; 66(4):422-30.HI

Abstract

Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder with laboratory findings similar to those for congenital von Willebrand disease. Unlike the congenital form, AVWS usually occurs in individuals with no personal or family history of bleeding disorders. According to an international registry, AVWS is mainly associated with lymphomyeloproliferative, immunologic, and cardiovascular disorders, as well as with solid tumors and other miscellaneous conditions; however, the prevalence of AVWS in these underlying disorders is still unknown. von Willebrand factor (VWF) is synthesized normally in most AVWS patients, and the low plasma VWF levels are from its accelerated removal from plasma by five different mechanisms, including autoantibodies. Because of the reduced half-life of endogenous-exogenous plasma VWF, bleeding of AVWS cannot be managed with desmopressin or factor VIII/VWF concentrates. Clinical use of intravenous immunoglobulin (IVIg) in AVWS has been reported since 1988. IVIg is most effective in AVWS with type immunoglobulin (Ig) G monoclonal gammopathies of undetermined significance and in other cases with IgG autoantibodies. IVIg can correct factor VIII and von Willebrand factor complex activities for about 15-20 days, and repeated injections induce remission of AVWS in these patients. Prospective studies are required to evaluate the efficacy and safety of IVIg in AVWS.

Authors+Show Affiliations

Angelo Bianchi Bonomi Hemophilia Thrombosis Center, Department of Internal Medicine and Dermatology, IRCCS Maggiore Hospital and University of Milan, Milan, Italy. Augusto.Federici@unimi.it

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

15866707

Citation

Federici, Augusto B.. "Use of Intravenous Immunoglobulin in Patients With Acquired Von Willebrand Syndrome." Human Immunology, vol. 66, no. 4, 2005, pp. 422-30.
Federici AB. Use of intravenous immunoglobulin in patients with acquired von Willebrand syndrome. Hum Immunol. 2005;66(4):422-30.
Federici, A. B. (2005). Use of intravenous immunoglobulin in patients with acquired von Willebrand syndrome. Human Immunology, 66(4), 422-30.
Federici AB. Use of Intravenous Immunoglobulin in Patients With Acquired Von Willebrand Syndrome. Hum Immunol. 2005;66(4):422-30. PubMed PMID: 15866707.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Use of intravenous immunoglobulin in patients with acquired von Willebrand syndrome. A1 - Federici,Augusto B, PY - 2004/12/23/received PY - 2005/01/19/accepted PY - 2005/5/4/pubmed PY - 2006/6/16/medline PY - 2005/5/4/entrez SP - 422 EP - 30 JF - Human immunology JO - Hum. Immunol. VL - 66 IS - 4 N2 - Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder with laboratory findings similar to those for congenital von Willebrand disease. Unlike the congenital form, AVWS usually occurs in individuals with no personal or family history of bleeding disorders. According to an international registry, AVWS is mainly associated with lymphomyeloproliferative, immunologic, and cardiovascular disorders, as well as with solid tumors and other miscellaneous conditions; however, the prevalence of AVWS in these underlying disorders is still unknown. von Willebrand factor (VWF) is synthesized normally in most AVWS patients, and the low plasma VWF levels are from its accelerated removal from plasma by five different mechanisms, including autoantibodies. Because of the reduced half-life of endogenous-exogenous plasma VWF, bleeding of AVWS cannot be managed with desmopressin or factor VIII/VWF concentrates. Clinical use of intravenous immunoglobulin (IVIg) in AVWS has been reported since 1988. IVIg is most effective in AVWS with type immunoglobulin (Ig) G monoclonal gammopathies of undetermined significance and in other cases with IgG autoantibodies. IVIg can correct factor VIII and von Willebrand factor complex activities for about 15-20 days, and repeated injections induce remission of AVWS in these patients. Prospective studies are required to evaluate the efficacy and safety of IVIg in AVWS. SN - 0198-8859 UR - https://www.unboundmedicine.com/medline/citation/15866707/Use_of_intravenous_immunoglobulin_in_patients_with_acquired_von_Willebrand_syndrome_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0198-8859(05)00044-3 DB - PRIME DP - Unbound Medicine ER -
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