Ueno Risa, Kado Hiroshi, Shiotsu Yayoi, Hara Masayuki, Otani Mai, Segawa Hiroyoshi, Sawada Katsunori, Hatta Tsuguru
Department of Nephrology, Ohmihachiman Community Medical Center, Shiga, Japan.
Nihon Jinzo Gakkai Shi. 2012;54(5):629-36.
A 27-year-old woman was referred to our hospital because of pancytopenia and nephritic syndrome in November, 2008. The findings of physical and laboratory examinations showed systemic lupus erythematosus (SLE). Diffuse proliferative lupus nephritis(group IV-G(A))was confirmed by renal biopsy. After combined therapy with prednisolone, intravenous cyclophosphamide pulse and mizoribine, proteinuria decreased from 13.0 g/day to 2.0 g/day and the serum complement level recovered to the normal level. However, she visited our hospital again for management of bleeding tendency in July 2009. She was diagnosed as hemophagocytic syndrome (HPS), with pancytopenia, high ferritin, high LDH level and hemophagocytosis in the bone marrow. She was treated effectively with steroid pulse therapy, but relapsed with HPS after two weeks. Although her child caught a cold, the case did not show any sign or symptom of infection, such as the common cold. However, we diagnosed her HPS as infection-associated hemophagocytic syndrome (IAHS) because she was not in the active phase of SLE at the onset of hemophagocytosis and the laboratory findings showed elevation of her serum ferritin and LDH. Therefore, we considered that her infectious sign may have been concealed by immunosuppressive therapy with prednisolone for SLE. It is very difficult to distinguish between IAHS and autoimmune-associated hemophagocytic syndrome (AAHS)in autoimmune diseases, but the differential diagnosis is necessary to treat the HPS. Here, we report an important case of HPS complicated with SLE. This case may attract interest particularly in the management of HPS-complicated autoimmune disease. Therefore, we report it with a review of the literature.
2008年11月，一名27岁女性因全血细胞减少和肾病综合征转诊至我院。体格检查和实验室检查结果显示为系统性红斑狼疮（SLE）。肾活检确诊为弥漫性增殖性狼疮肾炎（IV - G(A)组）。在接受泼尼松龙、静脉注射环磷酰胺脉冲和米唑嘌呤联合治疗治疗后，蛋白尿从13.0克/天降至2.0克/天，血清补体水平恢复正常。然而，2009年7月她因出血倾向再次来我院就诊。她被诊断为噬血细胞综合征（HPS），伴有全血细胞减少、铁蛋白升高、乳酸脱氢酶水平升高以及骨髓噬血细胞现象。她接受了类固醇脉冲疗法，治疗有效，但两周后HPS复发。尽管她的孩子感冒了，但该病例未表现出任何感染迹象或症状，如普通感冒。然而，我们将她的HPS诊断为感染相关性噬血细胞综合征（IAHS），因为她在噬血细胞增多症发作时并非处于SLE活动期，且实验室检查结果显示其血清铁蛋白和乳酸脱氢酶升高。因此，我们认为她的感染迹象可能被用于治疗SLE的泼尼松龙免疫抑制疗法所掩盖。在自身免疫性疾病中，很难区分IAHS和自身免疫相关性噬血细胞综合征（AAHS），但鉴别诊断对于治疗HPS是必要的。在此，我们报告一例HPS合并SLE的重要病例。该病例可能特别引起对HPS合并自身免疫性疾病管理方面的关注。因此，我们结合文献回顾对其进行报告。
