Thomas Mathew, Robert Alex, Kuruvilla Neenu, C Uthamanand
Breast Medical Oncology, Cleveland Clinic, Cleveland, USA.
Internal Medicine, Church of South India (CSI) Holdsworth Memorial Hospital, Mysore, IND.
Cureus. 2019 Aug 19;11(8):e5427. doi: 10.7759/cureus.5427.
Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening hyper-inflammatory condition characterized by excessive activation of macrophages and T cells resulting in multi-organ dysfunction. HLH can be primary/familial or secondary to infections, malignancy, immunosuppression, and autoimmune conditions. Systemic lupus erythematosus (SLE) is an autoimmune condition that can predispose to HLH. SLE, as other immune conditions is more common in females than in males. However, the occurrence of SLE in males and subsequent predisposition to HLH is rare. We report the case of a 22-year-old gentleman who presented with fever for three months and one day of altered sensorium prior to admission. On evaluation, he fulfilled five out of the 17 diagnostic criteria for SLE. His bone marrow biopsy showed hemophagocytosis and met five out of the eight diagnostic criteria of HLH, and a diagnosis of HLH secondary to SLE was made. He was treated with pulse doses of intravenous methylprednisolone and azathioprine and showed dramatic improvement. A high index of suspicion is essential for the diagnosis of HLH and prompt initiation of treatment is of utmost importance for tackling such a rapidly progressive life-threatening condition.
噬血细胞性淋巴组织细胞增生症（HLH）是一种具有侵袭性且危及生命的高度炎症性疾病，其特征为巨噬细胞和T细胞过度活化，导致多器官功能障碍。HLH可为原发性/家族性，或继发于感染、恶性肿瘤、免疫抑制及自身免疫性疾病。系统性红斑狼疮（SLE）是一种可引发HLH的自身免疫性疾病。与其他免疫性疾病一样，SLE在女性中比在男性中更为常见。然而，男性发生SLE并随后易患HLH的情况较为罕见。我们报告一例22岁男性患者，入院前发热三个月，意识改变一天。经评估，他符合SLE 17项诊断标准中的5项。他的骨髓活检显示噬血细胞现象，符合HLH 8项诊断标准中的5项，最终诊断为继发于SLE的HLH。他接受了大剂量静脉注射甲泼尼龙和硫唑嘌呤治疗，病情显著改善。对于HLH的诊断，高度的怀疑指数至关重要，而迅速开始治疗对于应对这种迅速进展的危及生命的疾病至关重要。
