Gupta Devika, Mohanty Supreet, Thakral Deepshi, Bagga Arvind, Wig Naveet, Mitra Dipendra Kumar
Department of Transplant Immunology & Immunogenetics, All India Institute of Medical Sciences, New Delhi, India.
Department of Paediatrics, All India Institute of Medical Sciences, New Delhi, India.
Am J Case Rep. 2016 Oct 13;17:739-744. doi: 10.12659/ajcr.899433.
BACKGROUND Hemophagocytic lymphohistiocytosis (HLH) in the background of systemic lupus erythematosus (SLE) is rare. Inability to discriminate between these two entities may be fatal for the patient. Here we report two cases of SLE with secondary HLH, one of which manifested HLH as the initial presentation, and the significance of HLH's timely diagnosis. CASE REPORT We describe two cases of SLE secondarily affected by HLH, which were diagnosed by various laboratory parameters and detection of profoundly reduced NK cell activity by using flow cytometry. Both our cases on investigation showed hyperferritinemia, hypertriglyceridemia, hypofibrinogenemia, and marked reduction or complete absence of NK cell activity. CONCLUSIONS Association of secondary HLH with SLE is rare, and when it occurs, differentiating it from lupus flare requires a high degree of suspicion and awareness of this association. Both have overlapping clinical features, but HLH is characterized by hyperferritinemia, hypofibrinogenemia, hypertriglyceridemia, and a decrease in erythrocyte sedimentation rate (ESR) and NK cell activity unlike SLE. Therefore, early diagnosis of HLH in the background of SLE facilitates timely selection of an appropriate treatment modality to prevent fatal complications.
背景 系统性红斑狼疮（SLE）背景下的噬血细胞性淋巴组织细胞增生症（HLH）较为罕见。无法区分这两种疾病可能对患者致命。在此，我们报告两例继发HLH的SLE病例，其中一例以HLH为首发表现，并阐述HLH及时诊断的重要性。病例报告 我们描述两例继发HLH的SLE病例，通过各种实验室参数及利用流式细胞术检测到自然杀伤（NK）细胞活性显著降低而确诊。我们的两例病例在检查时均显示高铁蛋白血症、高甘油三酯血症、低纤维蛋白原血症以及NK细胞活性显著降低或完全缺失。结论 继发HLH与SLE的关联罕见，当其发生时，将其与狼疮活动相鉴别需要高度怀疑并认识到这种关联。两者具有重叠的临床特征，但HLH的特点是高铁蛋白血症、低纤维蛋白原血症、高甘油三酯血症以及红细胞沉降率（ESR）和NK细胞活性降低，这与SLE不同。因此，在SLE背景下早期诊断HLH有助于及时选择合适的治疗方式以预防致命并发症。
