From the Division of Rheumatology, Department of Internal Medicine, Bundang CHA, Medical Center, Seongnam, South Korea.
J Clin Rheumatol. 2013 Dec;19(8):449-51. doi: 10.1097/RHU.0000000000000040.
Hemophagocytic syndrome (HPS) is a rare but potentially fatal disease caused by the activation of benign macrophage in various organs including bone marrow and the mononuclear phagocytes system. Here we report a case of 23-year-old woman in whom HPS occurred along with lupus flare during follow-up after the diagnosis of systemic lupus erythematosus. The patient showed aggravated malar rash, discoid rash, febrile cytopenia, hyperferritinemia, and elevated liver enzymes. She underwent ultrasound-guided percutaneous gun biopsy of the liver using an 18-gauge needle. As activated macrophages with hemophagocytosis were confirmed in hepatic histology, the patient was diagnosed as having reactive HPS related to a lupus flare. Clinical symptoms and laboratory findings were improved through high-dose intravenous methylprednisolone therapy followed by oral prednisolone and cyclosporine maintenance therapy. In lupus patients with persistent high fever, cytopenia, elevated levels of serum ferritin, and liver enzymes, reactive HPS should be strongly suspected, and aggressive examinations such as liver biopsy need to be considered for early diagnosis and proper treatment.
噬血细胞综合征（HPS）是一种罕见但潜在致命的疾病，由骨髓和单核吞噬细胞系统等各种器官中的良性巨噬细胞激活引起。本文报告了一例 23 岁女性病例，该患者在系统性红斑狼疮诊断后随访期间狼疮发作时发生 HPS。患者表现为红斑加重、盘状皮疹、发热性血细胞减少症、高铁蛋白血症和肝酶升高。她接受了 18 号针经超声引导的肝穿刺活检。由于在肝组织学中证实存在具有噬血细胞作用的活化巨噬细胞，因此诊断为狼疮发作相关的反应性 HPS。通过大剂量静脉注射甲基强的松龙治疗，随后口服泼尼松龙和环孢素维持治疗，临床症状和实验室检查结果得到改善。对于持续性高热、血细胞减少症、血清铁蛋白和肝酶水平升高的狼疮患者，应强烈怀疑反应性 HPS，并考虑进行肝脏活检等积极检查，以实现早期诊断和适当治疗。
