Ly B, Michaelsen T E, Dahl O, Frøland S S
Scand J Haematol. 1982 Feb;28(2):132-40. doi: 10.1111/j.1600-0609.1982.tb00505.x.
A 73-year-old previously healthy woman was admitted because of severe bleeding from esophagitic lesions and intraabdominal bleeding following hysterectomy. Acquired hemophilia, probably due to an IgG antibody to factor VIII (64 inhibitor units/ml) was noticed, the VIII:C in the patient's plasma being 18% or normal. Immune complexes isolated by polyethylene glycol precipitation had only a weak factor VIII inhibiting activity whereas IgG purified from the complexes and monomeric IgG present in her plasma exerted a strong inhibition. Removal of the complexes from plasma had no effect on the inhibitor titer thus indicating that only a minor part of the antibody was circulating as immune complexes. Plasma or purified IgG from the patient decreased the VIII:C of normal plasma to 18 og 14%, respectively, total inhibition being impossible to achieve even in antibody excess, probably reflecting residual activity of factor VIII bound to the patient's antibodies. The ristocetin cofactor activity of normal plasma was unaffected by the antibodies. Transfusion of factor VIII concentrate to the patient resulted in therapeutic levels of circulating factor VIII and transfused factor VIII circulated longer than usual. Partial remission of the disease with adequate levels of VIII:C occurred after 3 months.
一名73岁既往健康的女性因食管病变严重出血及子宫切除术后腹腔内出血入院。发现其患有获得性血友病，可能是由于存在针对凝血因子VIII的IgG抗体（64抑制单位/毫升），患者血浆中的VIII:C为18%或正常水平。通过聚乙二醇沉淀分离出的免疫复合物仅具有较弱的凝血因子VIII抑制活性，而从复合物中纯化的IgG及其血浆中存在的单体IgG则具有较强的抑制作用。从血浆中去除复合物对抑制剂滴度无影响，这表明只有一小部分抗体以免疫复合物的形式循环。患者的血浆或纯化IgG分别将正常血浆的VIII:C降至18%或14%，即使在抗体过量的情况下也无法实现完全抑制，这可能反映了与患者抗体结合的凝血因子VIII的残余活性。正常血浆的瑞斯托霉素辅因子活性不受抗体影响。给患者输注凝血因子VIII浓缩物后，循环中的凝血因子VIII达到治疗水平，且输注的凝血因子VIII循环时间比平时更长。3个月后，随着VIII:C水平充足，疾病出现部分缓解。
