{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T14:51:10Z","timestamp":1790175070445,"version":"4.1.0"},"reference-count":14,"publisher":"SAGE Publications","issue":"3","license":[{"start":{"date-parts":[[1999,7,1]],"date-time":"1999-07-01T00:00:00Z","timestamp":930787200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Clin Appl Thromb Hemost"],"published-print":{"date-parts":[[1999,7]]},"abstract":"<jats:p>The reported underlying lymphoproliferative dis orders associated with acquired von Willebrand disease (AvWD) include benign monoclonal gammapathy, multiple myeloma, Waldenstr\u00f6m disease, chronic lymphocytic leuke mia, non-Hodgkin lymphoma, and hairy cell leukemia. The AvWD in patients with a monoclonal gammapathy and\/or a lymphoproliferative disorder is featured by a prolonged bleed ing time, normal platelet count, and a decreased or absent ris tocetine-induced platelet aggregation in combination with a prolonged aPTT and normal PT due to low levels of factor VIII\/von Willebrand factor (vWF) parameters in the absence of a factor VIII inhibitor in the Bethesda assay. In vitro and vivo experiments consistently showed that the anti-vWF autoanti bodies in monoclonal gammapathies cause a rapid clearance of the factor VIII\/vWF complex from the circulation after DDAVP and factor VIII\/vWF concentrate infusion. Multimeric analysis of the vWF usually show a type 11-like AvWD due to the absence of large vWF multimers as the consequence of the rapid clearance of the anti-vWF-factor VIII\/vWF complex from the circulation. There is a poor response to intravenous DDAVP and factor VIII\/vWF concentrate infusion, but high dose intravenous gamma globulin (1 g\/kg for 2 days) usually induces a transient correction of the factor VIII\/vWF param eters for 1 to a few weeks.<\/jats:p>","DOI":"10.1177\/107602969900500302","type":"journal-article","created":{"date-parts":[[2007,11,26]],"date-time":"2007-11-26T17:47:05Z","timestamp":1196099225000},"page":"152-157","source":"Crossref","is-referenced-by-count":18,"title":["Acquired von Willebrand Disease in Monoclonal Gammapathies: Effectiveness of High-dose Intravenous Gamma Globulin"],"prefix":"10.1177","volume":"5","author":[{"given":"Jan Jacques","family":"Michiels","sequence":"first","affiliation":[{"name":"Department of Clinical Hematology, Academic Medical Center, Amsterdam and Goodheart Institute, Rotterdam"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Huub H. D. 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