Dumoulin F L, Daniel W G, Lichtlen P, Schmidt R E
Abteilung Immunologie und Transfusionsmedizin, Zentrums Innere Medizin und Dermatologie der Medizinischen Hochschule Hannover.
Dtsch Med Wochenschr. 1993 Jun 18;118(24):903-6. doi: 10.1055/s-2008-1059403.
A 37-year-old woman in acute right heart failure had experienced systemic venous thromboses for 17 years, five miscarriages and repeated pulmonary emboli. For the last 7 years she had been treated symptomatically for pulmonary hypertension. The platelet count was 62,000/microliters, thromboplastin time under phenprocoumon was 22%, partial thromboplastin time was 72 s. Despite anticoagulation with phenprocoumon and heparin (7,500 IU two times daily subcutaneously) new pulmonary emboli occurred and platelet count fell to 12,000/microliters. An increased titre for anticardiolipin antibodies (IgG > 320 GPL U/l, IgM 8 MPL U/l), antinuclear (1:640) and anti-ds-DNA antibodies (> 200 IU/ml) with simultaneous complement consumption suggested secondary antiphospholipid syndrome associated with lupus erythematodes. Treatment with prednisolone (150 mg/d), immunoglobulins (20 mg/d intravenously for 5 days) and heparin (25,000 IU/24 h intravenously) achieved an increase in platelet count to 200,000/microliters within 10 days, but fell again when the prednisolone dose was reduced, recovering under azathioprine, 150 mg/d. Four weeks later the patient died of renewed acute right heart failure.
一名37岁急性右心衰竭女性有17年系统性静脉血栓形成病史，5次流产及反复肺栓塞。过去7年她一直针对肺动脉高压进行对症治疗。血小板计数为62,000/微升，苯丙香豆素治疗下的凝血酶原时间为22%，部分凝血活酶时间为72秒。尽管使用苯丙香豆素和肝素（每日2次皮下注射7,500国际单位）进行抗凝治疗，仍出现新的肺栓塞，血小板计数降至12,000/微升。抗心磷脂抗体（IgG > 320 GPL U/l，IgM 8 MPL U/l）、抗核抗体（1:640）及抗双链DNA抗体（> 200 IU/ml）滴度升高，同时伴有补体消耗，提示与红斑狼疮相关的继发性抗磷脂综合征。使用泼尼松龙（150毫克/天）、免疫球蛋白（静脉注射20毫克/天，共5天）及肝素（静脉注射25,000国际单位/24小时）治疗，10天内血小板计数升至200,000/微升，但泼尼松龙剂量减少时又再次下降，在硫唑嘌呤150毫克/天治疗下恢复。四周后患者死于再次发作的急性右心衰竭。
