Mannucci P M, Canciani M T, Mari D, Meucci P
Scand J Haematol. 1979 May;22(5):423-32. doi: 10.1111/j.1600-0609.1979.tb00440.x.
An acquired inhibitor of blood coagulation, similar to that described in patients with Systemic Lupus Erythematosus (SLE), was detected during routine coagulation screening in 10 patients who did not meet the criteria for a diagnosis of SLE. The lupus-like anticoagulant (LLAC) was diagnosed on the basis of prolonged activated partial thromboplastin time (APTT) and/or prothrombin time (PT) which failed to correct when patient plasma was added to normal plasma; an additional criterion was an abnormal tissue thromboplastin inhibition test. No patient had a specific inhibitor directed against factors VIII and IX. Demonstration of LLAC was highly dependent upon the type of reagents adopted in the APTT and PT: the abnormality was detected consistently by one reagent only. One-stage assays of factors VIII and IX were characteristic of the presence of an inhibitor, showing non-parellel dose-response curves or decreased activity at low dilutions which were partially corrected at higher dilutions. Although 7 patients were free of abnormal bleeding, unequivocal signs of haemorrhagic tendency after a surgery were present in the remaining 3 patients. The findings suggest that LLAC is a non-exceptional cause of prolonged coagulation screening tests, and that it may sometimes be associated with impaired haemostasis.
在10例不符合系统性红斑狼疮(SLE)诊断标准的患者进行常规凝血筛查时,检测到一种获得性凝血抑制剂,类似于SLE患者中所描述的情况。狼疮样抗凝物(LLAC)是根据活化部分凝血活酶时间(APTT)延长和/或凝血酶原时间(PT)延长来诊断的,当将患者血浆加入正常血浆时,这些指标未能得到纠正;另一个标准是组织凝血活酶抑制试验异常。没有患者有针对因子VIII和IX的特异性抑制剂。LLAC的检测高度依赖于APTT和PT中所采用的试剂类型:仅一种试剂能始终检测到异常。因子VIII和IX的一期检测具有抑制剂存在的特征,表现为非平行剂量反应曲线或在低稀释度时活性降低,在高稀释度时部分得到纠正。虽然7例患者没有异常出血,但其余3例患者在手术后有明确的出血倾向迹象。这些发现表明,LLAC是凝血筛查试验延长的一个常见原因,并且它有时可能与止血功能受损有关。