Kuruvilla Neenu, Rajendran Rahul, Thomas Shilpa S, Ali Km Irshad, Kurian Sheela
Department of Internal Medicine, Government Medical College, Kottayam, IND.
Cureus. 2020 Oct 24;12(10):e11139. doi: 10.7759/cureus.11139.
Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and potentially fatal condition characterized by immune activation and multi-organ dysfunction. HLH can be inherited in an autosomal recessive fashion, but can also be secondary to infections, malignancy, immunosuppression, and autoimmune conditions. Adult-onset Still's disease (AOSD) is an autoimmune disorder that can predispose patients to HLH. AOSD, similar to other autoimmune conditions, is more common in females than males. However, the occurrence of AOSD in males and subsequent predisposition to HLH is rarely reported. We report the case of a 23-year-old male patient who presented with fever, joint pain, and rash for 20 days. On evaluation, he fulfilled the diagnostic criteria for AOSD and HLH, and a diagnosis of HLH secondary to AOSD was made. He was treated with pulse dose steroids and gradually tapered. AOSD and HLH have overlapping clinical and laboratory features and hence their co-occurrence poses diagnostic challenges. The mortality rate of HLH is high and hence prompt initiation of treatment is of utmost importance.
噬血细胞性淋巴组织细胞增生症（HLH）是一种侵袭性且可能致命的疾病，其特征为免疫激活和多器官功能障碍。HLH可呈常染色体隐性遗传方式，但也可能继发于感染、恶性肿瘤、免疫抑制和自身免疫性疾病。成人斯蒂尔病（AOSD）是一种自身免疫性疾病，可使患者易患HLH。与其他自身免疫性疾病一样，AOSD在女性中比男性更常见。然而，男性AOSD的发生及随后易患HLH的情况鲜有报道。我们报告一例23岁男性患者，其发热、关节疼痛和皮疹20天。经评估，他符合AOSD和HLH的诊断标准，诊断为继发于AOSD的HLH。他接受了脉冲剂量类固醇治疗并逐渐减量。AOSD和HLH具有重叠的临床和实验室特征，因此它们的同时出现带来了诊断挑战。HLH的死亡率很高，因此及时开始治疗至关重要。
