{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T13:20:39Z","timestamp":1760188839920},"reference-count":26,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2006,7,11]],"date-time":"2006-07-11T00:00:00Z","timestamp":1152576000000},"content-version":"vor","delay-in-days":4089,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[1995,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>A new variant of von Willebrand's disease has been discovered in 2 members of a Macedonian family of 6. The proband, an 8\u2010year\u2010old boy, showed a prolonged bleeding episode on 1 occasion. Ristocetin\u2010induced platelet aggregation and bleeding time were normal. In plasma, ristocetin cofactor activity (RCo) and von Willebrand factor (vWf) antigen were reduced to the same clearly low level. The determination of vWf antigen of platelets resulted in borderline values, while RCo was clearly reduced. Low\u2010 and intermediate\u2010resolution agarose gel electrophoresis showed absence of the largest multimers in plasma vWf, and slight reduction in platelet vWf. High\u2010resolution gels revealed abnormal multimeric structure only in plasma vWf. The smaller multimers could be resolved in a broad central band flanked by 4 fainter satellite bands; however, satellite bands close to the central band were more intense, and more distant ones were fainter, compared to normal plasma. The central band of the fastest\u2010moving multimer was markedly intensified, and the mobility of the whole quintuplet was slightly reduced. Heredity seems to be autosomal\u2010dominant. No mutation was found in exon 28 of the vWf gene.<\/jats:p><jats:p>Because there was only 1 mild bleeding episode in the family, this structural variation seems to have only little clinical consequence. We conclude that this vWf abnormality is different from those observed in other type II variants previously described. Based on the revised classification by the International Society on Thrombosis and Haemostasis, we proposed designation type 2A\u2010Bern for this new subtype. \u00a9 1995 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajh.2830490105","type":"journal-article","created":{"date-parts":[[2007,2,21]],"date-time":"2007-02-21T13:55:04Z","timestamp":1172066104000},"page":"21-28","source":"Crossref","is-referenced-by-count":2,"title":["New variant of type II von willebrand's disease with structural abnormality of plasma von willebrand factor in a patient with very mild bleeding history"],"prefix":"10.1002","volume":"49","author":[{"given":"Peter","family":"Baillod","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christine","family":"Gaucher","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Beat","family":"Affolter","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claudine","family":"Mazurier","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rolf","family":"Pflugshaupt","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2006,7,11]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V70.4.895.895"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0025-6196(12)61204-X"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI109795"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI110700"},{"key":"e_1_2_1_6_2","doi-asserted-by":"crossref","first-page":"1369","DOI":"10.1182\/blood.V63.6.1369.1369","article-title":"A new variant of dominant type II von Willebrand's disease with aberrant multimeric pattern of factor VIII\u2010related antigen type IID)","volume":"63","author":"Kinoshita S","year":"1984","journal-title":"Blood"},{"key":"e_1_2_1_7_2","doi-asserted-by":"crossref","first-page":"1419","DOI":"10.1182\/blood.V69.5.1419.1419","article-title":"Type II A von Willebrand disease with apparent recessive inheritence","volume":"69","author":"Asakura A","year":"1987","journal-title":"Blood"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI112394"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830220311"},{"key":"e_1_2_1_10_2","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1182\/blood.V68.1.269.269","article-title":"A new variant of type II von Willebrand disease with aberrant multimeric structure of plasma but not platelet von Willebrand factor (type IIF)","volume":"68","author":"Mannucci PM","year":"1986","journal-title":"Blood"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830240305"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830320409"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/0049-3848(92)90165-7"},{"key":"e_1_2_1_14_2","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1182\/blood.V82.1.169.bloodjournal821169","article-title":"New variant of von Willebrand type II with markedly increased levels of von Willebrand factor antigen and dominant mode of inheritance: von Willebrand disease type IIC Miami","volume":"82","author":"Ledford MR","year":"1993","journal-title":"Blood"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1055\/s-0038-1651576"},{"key":"e_1_2_1_16_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(19)47144-5"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI115376"},{"key":"e_1_2_1_18_2","first-page":"306","article-title":"A method of assaying von Willebrand factor (ristocetin cofactor)","volume":"34","author":"McFarlane DE","year":"1975","journal-title":"Thromb Diath Haemorrh"},{"key":"e_1_2_1_19_2","doi-asserted-by":"publisher","DOI":"10.1016\/0003-2697(66)90246-6"},{"key":"e_1_2_1_20_2","first-page":"18","article-title":"Dosage de I'antig\u00e8ne li\u00e9 au facteur VIII par la technique ELISA. 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