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Abstract：
Hemophagocytic lymphohistiocytosis (HLH) is a severe and life-threatening hyperinflammatory condition characterized by excessive activation of macrophages and T cells and resulted in multi-organ dysfunction. HLH can be a primary disease or secondary to infections, malignancy, and some autoimmune diseases, including adult-onset Still\'s disease (AOSD) and systemic lupus erythematosus (SLE). However, it is rare for HLH to occur as a secondary condition to drug-induced lupus erythematosus (DILE). In this report, we present a case of HLH as an unusual complication during SLE treatment in a 31-year-old male patient. The patient initially suffered from active chronic hepatitis B (CHB) and was treated with pegylated INFα-2b (Peg-INFα-2b), tenofovir disoproxil and lamivudine. After 19 months, CHB obtained biochemical and virological response with HBsAg positive to HBsAb. The patient developed fever, headache, and cytopenia after Peg-INFα-2b treatment for 33 months, and laboratory studies revealed that ANA and anti dsDNA were positive. He displayed 5 features meeting the HLH-2004 criteria for diagnosis including fever, pancytopenia, hyperferritinemia, high levels of soluble CD25, and hemophagocytosis on bone marrow biopsy. The patient was initiated with a combination treatment of intravenous methylprednisolone pulse therapy, oral cyclosporine, and etoposide (VP-16), which was followed by a course of oral prednisolone, intravenous cyclophosphamide pulse therapy, and entecavir with complete response. To our knowledge, this is the first report of IFN-α induced SLE complicating with HLH. Physicians should consider the potential autoimmune side effects of IFN-α therapy and be alert to insidious HLH in patients diagnosed with SLE.
摘要：
噬血细胞性淋巴组织细胞增生症（HLH）是一种严重且危及生命的高炎症状态，其特征是巨噬细胞和T细胞的过度激活并导致多器官功能障碍。HLH可以是原发性疾病或继发于感染，恶性肿瘤，和一些自身免疫性疾病，包括成人发作的斯蒂尔病（AOSD）和系统性红斑狼疮（SLE）。然而,HLH很少作为药物性红斑狼疮(DILE)的继发性疾病发生.在这份报告中,我们介绍了一例31岁男性患者在SLE治疗过程中出现HLH的异常并发症。患者最初患有活动性慢性乙型肝炎（CHB），并接受聚乙二醇化INFα-2b（PEG-INFα-2b）治疗，替诺福韦酯和拉米夫定。19个月后,CHB获得了HBsAg对HBsAb阳性的生化和病毒学应答。病人出现发热,头痛,Peg-INFα-2b治疗33个月后出现血细胞减少，实验室研究显示ANA和抗dsDNA呈阳性。他展示了5个符合HLH-2004诊断标准的特征，包括发烧，全血细胞减少症,高铁蛋白血症,高水平的可溶性CD25和骨髓活检的吞噬作用。患者开始接受静脉注射甲泼尼龙冲击治疗的联合治疗，口服环孢菌素,和依托泊苷（VP-16），随后是一个疗程的口服泼尼松龙，静脉注射环磷酰胺脉冲治疗，和恩替卡韦完全反应。据我们所知,这是IFN-α诱导的SLE并发HLH的首次报道。医师应考虑IFN-α治疗的潜在自身免疫副作用，并警惕诊断为SLE的患者的隐性HLH。
