PMID: 2120689Jul 1, 1990Paper

Acquired von Willebrand disease associated with free lambda light chain monoclonal gammopathy, normal bleeding time and response to prednisone

Postgraduate Medical Journal
A K Stewart, M F Glynn

Abstract

We report a case of acquired von Willebrand's syndrome with severe gastrointestinal bleeding and associated free monoclonal lambda light chains. The patient had a rapid sustained clinical and laboratory response to the administration of prednisone. Of note in this patient was the occurrence of angiodysplasia which has previously been reported in association with acquired von Willebrand's syndrome. No inhibitors of VWF:Ag, VWF:RCoF, or factor VIII:C were detected by mixing studies and the bleeding time was normal. Very few high molecular weight von Willebrand multimers were present prior to prednisone; however, the pattern reverted to a normal distribution following treatment. In appropriate patients with acquired von Willebrand's syndrome and monoclonal para-proteins, a trial of prednisone may be indicated.

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Citations

Apr 1, 1996·Medical and Pediatric Oncology·A B Jonge Poerink-StockschladerK Hählen
Oct 23, 2003·Transfusion Medicine Reviews·Theodore E WarkentinDavid G Morgan
Nov 16, 2012·The New England Journal of Medicine·Joseph Loscalzo
Nov 4, 2000·Expert Opinion on Investigational Drugs·A B Federici
Feb 11, 2004·The American Journal of the Medical Sciences·Yi-Wu Huang, Parvin Saidi
Mar 31, 1999·Baillière's Clinical Haematology·P J van Genderen, J J Michiels
Jan 16, 1998·The American Journal of Medicine·A Tefferi, W L Nichols
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Feb 20, 1997·Annals of the New York Academy of Sciences·N Delvaux, D Razavi
Dec 3, 1999·Haemophilia : the Official Journal of the World Federation of Hemophilia·I C Nitu-Whalley, C A Lee
Jul 11, 2006·American Journal of Hematology·Hiroshi Mohri
Nov 30, 2006·American Journal of Hematology·Massimo Franchini, Giuseppe Lippi
Mar 17, 2007·Critical Reviews in Clinical Laboratory Sciences·Massimo Franchini, Giuseppe Lippi

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