History

A 50-year-old woman presented to the emergency department of our hospital with a 2-day history of lower limb pain associated with unusual asthenia and diffuse arthralgia over the past 3 weeks. She was a native of Guinea and had lived in France for most of her life, working as a personal care assistant. Her only medical history of note was an occurrence of fetal death at 12 weeks gestation when she was 35 years old. She had bilateral lower limb swelling, without changes in skin temperature or color. All proximal and distal arterial pulses were felt. General physical examination findings were otherwise unremarkable.

Her laboratory tests showed a decreased hemoglobin concentration of 8.9 g/dL (normal range, 12–16 g/dL), a decreased platelet count of 45 × 10⁹/L (normal range, [150–400] × 10⁹/L), a C-reactive protein level of 158 mg/L (normal range, <5 mg/L), and a d-dimer level of 2000 mg/L (normal range, <500 mg/L]). Compression US of the lower limbs revealed bilateral calf vein thrombosis involving the fibular and posterior tibial veins. Curative anticoagulation using low-molecular-weight heparin (enoxaparin, subcutaneous injection of 100 units per kilogram of body weight twice a day) was started.

The day after the start of anticoagulation therapy, the patient reported dyspnea and acute chest and abdominal pain. Her vital signs were assessed, and she had elevated blood pressure and increased heart rate and respiratory rate, but she remained afebrile. Her cardiac auscultation was unremarkable, besides tachycardia. Skin examination revealed small areas of necrosis on the fingertips of her right hand. Laboratory studies were repeated and showed an increase in serum creatinine level from a baseline value of 0.49 mg/dL to a new value of 1.01 mg/dL (normal range, 0.6–1.1 mg/dL), an apparition of low-grade proteinuria of 0.43 g per day (normal range, <0.3 g/ day), and a high serum troponin level of 1066 ng/L (normal range, <14 ng/L), whereas electrocardiography showed no ST segment modification and echocardiography revealed a moderately altered left ventricular ejection fraction (45%). There was no coronary occlusion seen at emergency coronarography. Contrast-enhanced CT of the chest, abdomen, and pelvis was performed (Figs 1, 2) together with cardiac MRI (Figs 3, 4).

中文翻译：

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病例294：灾难性抗磷脂综合征

历史

一名 50 岁女性，在过去 3 周内出现下肢疼痛 2 天，伴有异常虚弱和弥漫性关节痛，就诊于我们医院的急诊科。她是几内亚人，一生中的大部分时间都住在法国，担任个人护理助理。她唯一值得注意的病史是在她 35 岁时在妊娠 12 周时发生胎儿死亡。她双下肢肿胀，皮肤温度或颜色没有变化。感觉到所有近端和远端动脉搏动。其他一般体格检查结果无异常。

她的实验室检查显示血红蛋白浓度降低 8.9 g/dL（正常范围，12-16 g/dL），血小板计数减少 45 × 10 ⁹ /L（正常范围，[150-400] × 10 ⁹ /L） )，C-反应蛋白水平为 158 mg/L（正常范围，<5 mg/L），d-二聚体水平为 2000 mg/L（正常范围，<500 mg/L]）。下肢加压超声显示双侧小腿静脉血栓形成，累及腓骨和胫后静脉。开始使用低分子量肝素（依诺肝素，皮下注射每公斤体重 100 单位，每天两次）进行治愈性抗凝治疗。

抗凝治疗开始后的第二天，患者报告呼吸困难和急性胸腹痛。对她的生命体征进行了评估，血压升高，心率和呼吸频率加快，但仍不发热。除了心动过速外，她的心脏听诊并无异常。皮肤检查显示她右手指尖有小块坏死。重复实验室研究并显示血清肌酐水平从基线值 0.49 mg/dL 增加到新值 1.01 mg/dL（正常范围，0.6-1.1 mg/dL），这是一种低度蛋白尿每天 0.43 g（正常范围，<0.3 g/天），以及 1066 ng/L 的高血清肌钙蛋白水平（正常范围，<14 ng/L），而心电图显示没有 ST 段改变，超声心动图显示左心室射血分数中度改变 (45%)。急诊冠状动脉造影未见冠状动脉闭塞。对胸部、腹部和骨盆进行了增强 CT（图 1、2）和心脏 MRI（图 3、4）。