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The antiphospholipid syndrome is an enigmatic disorder and this is reinforced by the misleading associated terminology, the adoption of which results directly from early discoveries relating to the condition. Thus the target antigen of the causative autoantibodies in antiphospholipid syndrome does not reside on phospholipid, and the frequently associated lupus anticoagulant is not restricted to subjects with systemic lupus erythematosus and, paradoxically, despite causing prolongation of clotting times <jats:italic>in vitro<\/jats:italic> it is associated with a pronounced tendency to thrombosis. Recognition of the antiphospholipid syndrome has its origins in the identification of subjects with so\u2010called biological false\u2010positive serological reactions for syphilis in the middle years of the last century. Since that time there have been considerable advances in our understanding of the pathogenesis of the disease and the clinical manifestations and associations, improved diagnostic accuracy and an evolving evidence base for optimal therapy. However many gaps in our knowledge remain.<\/jats:p>","DOI":"10.1111\/bjh.12848","type":"journal-article","created":{"date-parts":[[2014,3,29]],"date-time":"2014-03-29T06:15:37Z","timestamp":1396073737000},"page":"609-617","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["The chequered history of the antiphospholipid syndrome"],"prefix":"10.1111","volume":"165","author":[{"given":"Deepa","family":"Jayakody Arachchillage","sequence":"first","affiliation":[{"name":"Haemostasis Research Unit Department of Haematology University College London  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mike","family":"Greaves","sequence":"additional","affiliation":[{"name":"School of Medicine &amp; Dentistry University of Aberdeen Aberdeen UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,3,29]]},"reference":[{"key":"e_1_2_13_2_1","first-page":"1181","article-title":"Studies on the coagulation defect in a case of thrombocytopenic purpura complicated by thrombosis","volume":"22","author":"Aggeler P.M.","year":"1946","journal-title":"American Journal of Pathology"},{"key":"e_1_2_13_3_1","first-page":"482","article-title":"Primary antiphospholipid syndrome","volume":"16","author":"Alarcon\u2010Segovia D.","year":"1989","journal-title":"The Journal of Rheumatology"},{"key":"e_1_2_13_4_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0037-1614977"},{"key":"e_1_2_13_5_1","first-page":"1742","article-title":"A \u201cprimary\u201d antiphospholipid syndrome?","volume":"15","author":"Asherson R.A.","year":"1988","journal-title":"The Journal of Rheumatology"},{"key":"e_1_2_13_6_1","first-page":"508","article-title":"The catastrophic antiphospholipid syndrome","volume":"19","author":"Asherson R.A.","year":"1992","journal-title":"The Journal of Rheumatology"},{"key":"e_1_2_13_7_1","doi-asserted-by":"publisher","DOI":"10.1177\/014107688708000716"},{"key":"e_1_2_13_8_1","first-page":"568","article-title":"Disseminated intravascular coagulation, antiphospholipid antibodies and ischemic necrosis of the extremities","volume":"56","author":"Bird A.G.","year":"1987","journal-title":"Annals of the Rheumatic Diseases"},{"key":"e_1_2_13_9_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.88.8.3069"},{"key":"e_1_2_13_10_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.287.6398.1021"},{"key":"e_1_2_13_11_1","first-page":"416","article-title":"Thrombosis in systemic lupus erythematosus despite circulating anticoagulants","volume":"62","author":"Bowie E.J.","year":"1963","journal-title":"Journal of Laboratory and Clinical Medicine"},{"key":"e_1_2_13_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9378(12)90915-1"},{"key":"e_1_2_13_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9378(11)90700-5"},{"key":"e_1_2_13_14_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.1.5744.312"},{"key":"e_1_2_13_15_1","first-page":"2","article-title":"A hemorrhagic disorder caused by circulating anticoagulant in patients with disseminated lupus erythematosus","volume":"31","author":"Conley C.","year":"1952","journal-title":"Journal of Clinical Investigation"},{"key":"e_1_2_13_16_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa035241"},{"key":"e_1_2_13_17_1","doi-asserted-by":"publisher","DOI":"10.1177\/0961203312437810"},{"key":"e_1_2_13_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.22663"},{"key":"e_1_2_13_19_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.37759"},{"key":"e_1_2_13_20_1","first-page":"48","article-title":"Modification of trans\u2010operative changes in blood fluidity by hydroxy\u2010chloroquine. 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