病例报告:狼疮膜性肾病孕妇血栓性血小板减少性紫癜:诊断难题

Marina Leiva, Gustavo Navarro, J. D. Carpio, Leopoldo Ardiles
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摘要

一名怀孕 20 周的 27 岁女性因水肿和泡沫尿入院,但血压正常。她的血细胞计数正常,蛋白尿 3 克/天,肌酐 0.4 毫克/分升,白蛋白 2.4 克/分升,胆固醇 355 毫克/分升。抗核抗体为 1/160,但抗 DNA、抗心磷脂抗体和狼疮抗凝物均为阴性,血清 C3 和 C4 正常。肾活检显示继发性膜性肾小球病变,很可能是五级纯狼疮。开始使用类固醇、硫唑嘌呤和阿司匹林,直到怀孕 28 周时,她出现了严重的高血压、畏光、头痛、贫血、四肢广泛瘀伤、严重贫血、血小板减少,血肌酐升至 2.09 mg/dl,但利尿作用未受影响。一名重 1045 克的女婴通过紧急剖腹产娩出。手术后,她出现了复视、构音障碍、迟缓性精神障碍和下肢感觉改变,由于肺充血,不得不进行紧急血液透析。血涂片显示有血吸虫,LDH升高至1148 IU/L,而转氨酶和肝功能保持正常,提示血栓性血小板减少性紫癜。ADAMTS13的活性为6%,存在抑制剂。霉酚酸盐和每日血浆置换新鲜冰冻血浆的治疗效果并不理想,而甲基强的松龙脉冲和利妥昔单抗的使用也未见效果。最后,患者开始静脉注射环磷酰胺,结果血液病完全缓解,ADAMTS13也恢复正常,但透析依赖依然存在,四年后,右肾癌促使患者进行了双侧肾切除术。在接受泼尼松和羟氯喹治疗的六年后,她仍然没有肿瘤复发和狼疮活动。本文讨论了妊娠期狼疮患者微血管病变综合征的鉴别诊断。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Case report: Thrombotic thrombocytopenic purpura in a pregnant woman with lupus membranous nephropathy: a diagnostic challenge
A 27-year-old female at 20th week of pregnancy was admitted with edema, foamy urine, but normal blood pressure. Her blood count was normal, she had proteinuria of 3 g/day, creatinine 0.4 mg/dl, albumin 2.4 g/dl, and cholesterol 355 mg/dl. Antinuclear antibodies 1/160, but Anti-DNA, anticardiolipin antibodies and lupus anticoagulant were negative, with normal serum C3 and C4. A renal biopsy showed secondary membranous glomerulopathy, most likely lupus class V pure. Steroids, azathioprine, and aspirin were initiated, up to 28 weeks of pregnancy, when she developed severe hypertension, photopsia, headache, anasarca, extensive bruising of the extremities, severe anemia, thrombocytopenia, and creatinine rose to 2.09 mg/dl with preserved diuresis. A female infant, 1045 grams, was delivered by emergency caesarean section. Following the surgery, she experienced diplopia, dysarthria, bradypsychia, and sensory alterations in the lower extremities, necessitating emergency hemodialysis due to pulmonary congestion. Blood smear revealed schistocytes, LDH elevated at 1148 IU/L, while transaminases and liver function remained normal, suggesting thrombotic thrombocytopenic purpura. ADAMTS13 revealed 6% activity with the presence of inhibitor. Mycophenolate and daily plasmapheresis with fresh frozen plasma replacement yielded unsatisfactory response, unaffected by the addition of methylprednisolone pulses and rituximab. Eventually, intravenous cyclophosphamide was introduced, resulting in complete hematological remission and normalization of ADAMTS13, however dialysis-dependence persisted and four years later, right renal cancer prompted bilateral nephrectomy. After a total follow-up of six years, she remained free of neoplastic recurrence and lupus activity, receiving prednisone and hydroxychloroquine. The differential diagnosis of microangiopathic syndrome in a pregnant lupus patient is discussed.
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