{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,2,21]],"date-time":"2025-02-21T19:02:19Z","timestamp":1740164539539,"version":"3.37.3"},"reference-count":10,"publisher":"Wiley","license":[{"start":{"date-parts":[[2018,1,1]],"date-time":"2018-01-01T00:00:00Z","timestamp":1514764800000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100004702","name":"Baxter","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100004702","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100008322","name":"CSL Behring","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008322","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Case Reports in Hematology"],"published-print":{"date-parts":[[2018]]},"abstract":"<jats:p>We present a rare case of acquired von Willebrand syndrome (AVWS) caused by a mantle cell lymphoma. A 61-year-old male suffered from recurrent bleeding symptoms since a few months. Initially, physical examination was normal. von Willebrand factor antigen (VWF:Ag) level and factor VIII activity (FVIII:C) were low (0.31\u2009IU\/ml and 0.43\u2009IU\/ml, resp.). Ristocetin cofactor activity (VWF:RCo) was 0.09\u2009IU\/ml, and collagen binding activity (VWF:CB) was 0.10\u2009IU\/ml. VWF:RCo\/VWF:Ag ratio was 0.29, and RIPA value was normal. Highest molecular weight VWF multimers were absent. A diagnosis of von Willebrand Disease (VWD) type 2A was made. However, no genetic mutation was found. No inhibitory antibodies against VWF or factor VIII were detected. A few months later, cervical, axillary, and inguinal lymphadenopathy was found on physical examination. A CT scan confirmed multiple enlarged lymph nodes, and a clonal B-cell population matching a mantle cell lymphoma was detected in the bone marrow. Chemoimmunotherapy resulted in a very good partial remission and concomitantly in a rapid decrease of bleeding problems and complete normalization of FVIII:C and VWF:Ag. The diagnosis of AVWS cannot be rejected by negative mixing studies due to difficulties in the detection of autoantibodies and because of a highly heterogeneous pathogenesis of AVWS. When the suspicion of AVWS is high, an extensive investigation should be performed to find the underlying cause.<\/jats:p>","DOI":"10.1155\/2018\/7973297","type":"journal-article","created":{"date-parts":[[2018,1,30]],"date-time":"2018-01-30T18:31:57Z","timestamp":1517337117000},"page":"1-3","source":"Crossref","is-referenced-by-count":1,"title":["Acquired von Willebrand Disease Associated with Mantle Cell Lymphoma"],"prefix":"10.1155","volume":"2018","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1169-4354","authenticated-orcid":true,"given":"Dominique","family":"Maas","sequence":"first","affiliation":[{"name":"Department of Hematology, Radboud University Medical Center, Nijmegen, Netherlands"},{"name":"Hemophilia Treatment Center, Radboud University Medical Center, Nijmegen, Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Britta","family":"Laros-van Gorkom","sequence":"additional","affiliation":[{"name":"Department of Hematology, Radboud University Medical Center, Nijmegen, Netherlands"},{"name":"Hemophilia Treatment Center, Radboud University Medical Center, Nijmegen, Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sanne","family":"Gianotten","sequence":"additional","affiliation":[{"name":"Department of Hematology, Radboud University Medical Center, Nijmegen, Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marjan","family":"Cruijsen","sequence":"additional","affiliation":[{"name":"Department of Hematology, Radboud University Medical Center, Nijmegen, Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Waander","family":"van Heerde","sequence":"additional","affiliation":[{"name":"Hemophilia Treatment Center, Radboud University Medical Center, Nijmegen, Netherlands"},{"name":"Department of Laboratory Medicine, Laboratory for Hematology, Radboud University Medical Center, Nijmegen, Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marten","family":"Nijziel","sequence":"additional","affiliation":[{"name":"Department of Hematology, Radboud University Medical Center, Nijmegen, Netherlands"},{"name":"Hemophilia Treatment Center, Radboud University Medical Center, Nijmegen, Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","reference":[{"key":"1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-11-297580"},{"issue":"2","key":"2","first-page":"345","volume":"84","year":"2000","journal-title":"Thrombosis and Haemostasis"},{"key":"3","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2011.02681.x"},{"issue":"4","key":"4","first-page":"210","volume":"52","year":"2011","journal-title":"Rinsho Ketsueki"},{"key":"5","doi-asserted-by":"publisher","DOI":"10.1055\/s-0033-1334867"},{"key":"6","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.1997.d01-2088.x"},{"key":"7","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830350210"},{"key":"8","doi-asserted-by":"publisher","DOI":"10.1056\/nejm197902223000806"},{"key":"9","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1992.tb04562.x"},{"key":"10","doi-asserted-by":"publisher","DOI":"10.4065\/77.12.1391"}],"container-title":["Case Reports in Hematology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/downloads.hindawi.com\/journals\/crihem\/2018\/7973297.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/downloads.hindawi.com\/journals\/crihem\/2018\/7973297.xml","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/downloads.hindawi.com\/journals\/crihem\/2018\/7973297.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2018,5,18]],"date-time":"2018-05-18T12:27:00Z","timestamp":1526646420000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.hindawi.com\/journals\/crihem\/2018\/7973297\/"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018]]},"references-count":10,"alternative-id":["7973297","7973297"],"URL":"https:\/\/doi.org\/10.1155\/2018\/7973297","relation":{},"ISSN":["2090-6560","2090-6579"],"issn-type":[{"type":"print","value":"2090-6560"},{"type":"electronic","value":"2090-6579"}],"subject":[],"published":{"date-parts":[[2018]]}}}