Ames Paul R J, Graf Maria, Archer Jeremy, Scarpato Nicola, Iannaccone Luigi
Haemostasis & Thrombosis Department, William Harvey Research Institute, Charterhouse Square, London, United Kingdom Haemostasis & Thrombosis Department, St George's Hospital, London, United Kingdom
Dipartimento di Medicina Molecolare e Biotecnologie Mediche - Università degli Studi di Napoli Federico II - VIa Pansini, Napoli, Italy.
Clin Appl Thromb Hemost. 2015 Mar;21(2):149-54. doi: 10.1177/1076029614541516. Epub 2014 Jul 2.
To review the diagnostic difficulties of a prolonged activated partial thromboplastin time (aPTT) when 2 inhibitors with opposite clinical presentations coexist, we searched MEDLINE from January 1970 to November 2013 using acquired, factor VIII (FVIII), factor IX, hemophilia A and B, inhibitor, lupus anticoagulant (LA), antiphospholipid, anticardiolipin, anti-β2-glycoprotein I, antibodies, syndrome, bleeding, and thrombosis. We identified 13 articles for a total of 15 cases of possible coexistence of FVIII inhibitor and LA. The presenting clinical manifestation was thrombosis in 6 cases and bleeding in 9 cases. Activated partial thromboplastin time was the presenting laboratory abnormality in all cases, and first-line investigations suggested the coexistence of LA and acquired FVIII inhibitor. None of the articles addressed the diagnostic accuracy of the screening tests by performing "second line" assays. We reviewed the diagnostic pitfalls of the cases under study and provide some guidance for alternative tests when facing a prolonged aPTT that may have a double meaning.
为了探讨当两种临床表现相反的抑制剂共存时,活化部分凝血活酶时间(aPTT)延长的诊断难点,我们使用“获得性”“因子VIII(FVIII)”“因子IX”“血友病A和B”“抑制剂”“狼疮抗凝物(LA)”“抗磷脂”“抗心磷脂”“抗β2糖蛋白I”“抗体”“综合征”“出血”和“血栓形成”等关键词,检索了1970年1月至2013年11月的MEDLINE数据库。我们共筛选出13篇文章,其中共有15例可能同时存在FVIII抑制剂和LA的病例。临床表现为血栓形成的有6例,出血的有9例。所有病例的主要实验室异常均为活化部分凝血活酶时间延长,一线检查提示存在LA和获得性FVIII抑制剂。所有文章均未通过进行“二线”检测来探讨筛查试验的诊断准确性。我们回顾了所研究病例的诊断陷阱,并为面对可能具有双重意义的aPTT延长时的替代检测提供了一些指导。