{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,4]],"date-time":"2026-02-04T15:39:22Z","timestamp":1770219562405,"version":"3.49.0"},"reference-count":18,"publisher":"Oxford University Press (OUP)","issue":"777","license":[{"start":{"date-parts":[[1990,7,1]],"date-time":"1990-07-01T00:00:00Z","timestamp":646790400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[1990,7,1]]},"abstract":"<jats:title>Summary<\/jats:title>\n               <jats:p>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.<\/jats:p>\n               <jats:p>In appropriate patients with acquired von Willebrand's syndrome and monoclonal para-proteins, a trial of prednisone may be indicated.<\/jats:p>","DOI":"10.1136\/pgmj.66.777.560","type":"journal-article","created":{"date-parts":[[2008,12,29]],"date-time":"2008-12-29T21:01:03Z","timestamp":1230584463000},"page":"560-562","source":"Crossref","is-referenced-by-count":19,"title":["Acquired von Willebrand disease associated with free lambda light chain monoclonal gammopathy, normal bleeding time and response to prednisone"],"prefix":"10.1093","volume":"66","author":[{"given":"A K","family":"Stewart","sequence":"first","affiliation":[{"name":"Department of Medicine, Division of Haematology\/Oncology, Toronto General Hospital, University of Toronto , Toronto, Ontario, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M F X","family":"Glynn","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Haematology\/Oncology, Toronto General Hospital, University of Toronto , Toronto, Ontario, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[1990,7,1]]},"reference":[{"key":"ref_1","first-page":"83","article-title":"Hemostasis and Thrombosis. Basic Principles and Clinical Practice","author":"Colman, R.W.; Hirsh, J.; Marder, V.T.; Salzman, E.W.","year":"1987","unstructured":"Colman, R.W., Hirsh, J., Marder, V.T. & Salzman, E.W. Hemostasis and Thrombosis. Basic Principles and Clinical Practice, 2nd Edition. J.B. Lippincott, Philadelphia, 1987, pp. 83-84.","edition":"2"},{"issue":"112","key":"ref_2","first-page":"143","article-title":"The use of high dose intravenous globulin in acquired von Willebrand syndrome","volume":"1988","author":"Macik, B.G.; Gabriel, D.A.; White, G.C.; High, K.; Roberts, H.","unstructured":"Macik, B.G., Gabriel, D.A., White, G.C., High, K. & Roberts, H. The use of high dose intravenous globulin in acquired von Willebrand syndrome. Arch Pathol Lab Med 1988, 112: 143-146.","journal-title":"Arch Pathol Lab Med"},{"issue":"64","key":"ref_3","first-page":"614","article-title":"Studies ofthe pathophysiology of acquired von Willebrand's disease in seven patients with lymphoproliferative or benign monoclonal gammopathies","volume":"1984","author":"Mannucci, P.M.; Lombardi, R.; Bader, R.","unstructured":"Mannucci, P.M., Lombardi, R., Bader, R. et al. Studies ofthe pathophysiology of acquired von Willebrand's disease in seven patients with lymphoproliferative or benign monoclonal gammopathies. Blood 1984, 64: 614-621.","journal-title":"Blood"},{"issue":"59","key":"ref_4","first-page":"149","article-title":"IgA inhibitor to factor VIII\/von Willebrand factor","volume":"1985","author":"Gralnick, H.R.; Flaum, M.A.; Kessler, C.M.; Zimbler, H.; Coller, B.S.","unstructured":"Gralnick, H.R., Flaum, M.A., Kessler, C.M., Zimbler, H. & Coller, B.S. IgA inhibitor to factor VIII\/von Willebrand factor. Br J Haematol 1985, 59: 149-158.","journal-title":"Br J Haematol"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1030","DOI":"10.1136\/jcp.32.10.1030","article-title":"Acquired von Willebrand syndrome with IgM inhibitor against von Willebrand's factor","volume":"32","author":"Gouault-Heilmann, M.; Dumont, M.D.; Intrator, L.; Chenal, C.; Lejonc, J.L.","year":"1979","unstructured":"Gouault-Heilmann, M., Dumont, M.D., Intrator, L., Chenal, C. & Lejonc, J.L. Acquired von Willebrand syndrome with IgM inhibitor against von Willebrand's factor. J Clin Pathol 1979, 32: 1030-1035.","journal-title":"J Clin Pathol"},{"issue":"58","key":"ref_6","first-page":"665","article-title":"Acquired von Willebrand's disease in association with Wilms' tumour: regression following treatment","volume":"1981","author":"Scott, J.P.; Montgomery, R.R.; Tubergen, D.G.; Hay, T.","unstructured":"Scott, J.P., Montgomery, R.R., Tubergen, D.G. & Hay, T. Acquired von Willebrand's disease in association with Wilms' tumour: regression following treatment. Blood 1981, 58: 665.","journal-title":"Blood"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1111\/j.1365-2141.1983.tb02091.x","article-title":"Acquired von Willebrand's disease: demonstration of a circulating inhibitor to the factor VIII complex in four cases","volume":"54","author":"Sampson, B.M.; Greaves, M.; Malia, R.G.; Preston, F.E.","year":"1983","unstructured":"Sampson, B.M., Greaves, M., Malia, R.G. & Preston, F.E. Acquired von Willebrand's disease: demonstration of a circulating inhibitor to the factor VIII complex in four cases. Br J Haematol 1983, 54: 233-244.","journal-title":"Br J Haematol"},{"key":"ref_8","first-page":"412","article-title":"Production site of bleeding factor (acquired morbus von Willebrand)","volume":"23","author":"true","year":"1970","unstructured":"Velt kamp, J.J., Stevens, P., v.d.Plas, M. et al. Production site of bleeding factor (acquired morbus von Willebrand). Thrombos Diathes Haemorrh 1970, 23: 412.","journal-title":"Thrombos Diathes Haemorrh"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1111\/j.1365-2141.1980.tb03821.x","article-title":"Acquired von Willebrand's syndrome","volume":"45","author":"Holland, L.; Adamson, A.; Ingram, G.I.C.; Chalmers, D.G.","year":"1980","unstructured":"Holland, L., Adamson, A., Ingram, G.I.C. & Chalmers, D.G. Acquired von Willebrand's syndrome. Br J Haematol 1980, 45: 161-164.","journal-title":"Br J Haematol"},{"issue":"72","key":"ref_10","first-page":"303","article-title":"Acquired von Willebrand's disease due to hyaluronic acid produced by a hepatoma","volume":"1988","author":"Messmore, H.L.; Griffin, B.; Fareed, J.; Orfei, E.","unstructured":"Messmore, H.L., Griffin, B., Fareed, J. & Orfei, E. Acquired von Willebrand's disease due to hyaluronic acid produced by a hepatoma. Blood 1988, 72: 303a.","journal-title":"Blood"},{"issue":"54","key":"ref_11","first-page":"188","article-title":"A decrease in VIII: RCoF and larger multimers in myeloproliferative disease: relationship with bleeding diathesis","volume":"1985","author":"Fabris, F.; Casonato, A.; DeMarrco, L.; Mazzucato, M.; Girolaris, A.","unstructured":"Fabris, F., Casonato, A., DeMarrco, L., Mazzucato, M. & Girolaris, A. A decrease in VIII:RCoF and larger multimers in myeloproliferative disease: relationship with bleeding diathesis. Thromb Haemost 1985, 54: 188.","journal-title":"Thromb Haemost"},{"issue":"64","key":"ref_12","first-page":"981","article-title":"Acquired von Willebrand's disease in the myeloproliferative syndrome","volume":"1984","author":"Budde, U.; Schaefer, G.; Mueller, N.","unstructured":"Budde, U., Schaefer, G., Mueller, N. et al. Acquired von Willebrand's disease in the myeloproliferative syndrome. Blood 1984, 64: 981-985.","journal-title":"Blood"},{"key":"ref_13","first-page":"562","volume":"66","author":"Fricke, W.A.; Brinkhous, K.M.; Garris, J.B.; Roberts, H.R.","year":"1985","unstructured":"Fricke, W.A., Brinkhous, K.M., Garris, J.B. & Roberts, H.R. 1985, 66: 562-569."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"503","DOI":"10.1111\/j.1365-2141.1980.tb06001.x","article-title":"Acquired von Willebrand's syndrome associated with hairy cell leukaemia","volume":"46","author":"Roussi, J.M.; Houbouyan, L.L.; Alterescu, R.; Franc, B.; Goguel, A.F.","year":"1980","unstructured":"Roussi, J.M., Houbouyan, L.L., Alterescu, R., Franc, B. & Goguel, A.F. Acquired von Willebrand's syndrome associated with hairy cell leukaemia. Br J Haematol 1980, 46: 503-506.","journal-title":"Br J Haematol"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"408","DOI":"10.1056\/NEJM197902223000806","article-title":"A hemorrhagic syndrome in Waldenstrom's macroglobulinemia secondary to immunoabsorption of factor VIII, recovery after splenectomy","volume":"300","author":"Brody, J.I.; Haidar, M.E.; Rossman, R.E.","year":"1979","unstructured":"Brody, J.I., Haidar, M.E & Rossman, R.E. A hemorrhagic syndrome in Waldenstrom's macroglobulinemia secondary to immunoabsorption of factor VIII, recovery after splenectomy. N Engi J Med 1979, 300: 408-410.","journal-title":"N Engi J Med"},{"key":"ref_16","first-page":"285","article-title":"Selective absence of large forms of factor VIII\/von Willebrand factor in acquired von Willebrand's syndrome: response to transfusion","volume":"42","author":"Meyer, D.; Frommel, D.; Larrieu, M.J.; Zimmerman, T.S.","year":"1979","unstructured":"Meyer, D., Frommel, D., Larrieu, M.J., Zimmerman, T.S. et al. Selective absence of large forms of factor VIII\/von Willebrand factor in acquired von Willebrand's syndrome: response to transfusion. Thromb Haemost 1979, 42: 285.","journal-title":"Thromb Haemost"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"693","DOI":"10.1016\/0002-9343(79)90267-5","article-title":"Acquired von Willebrand disease associated with an inhibitor to factor VIII antigen and gastrointestinal telangiectasia","volume":"67","author":"McGrath, K.M.; Johnson, C.A.; Stuart, J.J.","year":"1979","unstructured":"McGrath, K.M., Johnson, C.A. & Stuart, J.J. Acquired von Willebrand disease associated with an inhibitor to factor VIII antigen and gastrointestinal telangiectasia. Am J Med 1979, 67: 693.","journal-title":"Am J Med"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1016\/0002-9343(78)90698-8","article-title":"Von Willebrand's disease, platelet release defect and angiodysplasia","volume":"65","author":"Rosborough, T.K.; Swaim, W.R.","year":"1978","unstructured":"Rosborough, T.K. & Swaim, W.R. Von Willebrand's disease, platelet release defect and angiodysplasia. Am J Med 1978, 65: 96-100.","journal-title":"Am J Med"}],"container-title":["Postgraduate Medical Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/pmj\/article-pdf\/66\/777\/560\/50077372\/postgradmedj-66-560.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/pmj\/article-pdf\/66\/777\/560\/50077372\/postgradmedj-66-560.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,4,28]],"date-time":"2023-04-28T14:19:34Z","timestamp":1682691574000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/pmj\/article\/66\/777\/560\/7061525"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1990,7,1]]},"references-count":18,"journal-issue":{"issue":"777","published-online":{"date-parts":[[1990,7,1]]},"published-print":{"date-parts":[[1990,7,1]]}},"URL":"https:\/\/doi.org\/10.1136\/pgmj.66.777.560","relation":{},"ISSN":["0032-5473","1469-0756"],"issn-type":[{"value":"0032-5473","type":"print"},{"value":"1469-0756","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[1990,7]]},"published":{"date-parts":[[1990,7,1]]}}}