Wang Gui, Jin Xiao-Rong, Jiang De-Xun
Beijing University of Chinese Medicine, Beijing 100029, China.
Rheumatism and Immunology Department, Seventh Medical Center of PLA General Hospital, Beijing 100700, China.
World J Clin Cases. 2020 Feb 6;8(3):560-567. doi: 10.12998/wjcc.v8.i3.560.
Adult-onset still disease (AOSD) and hemophagocytic syndrome (HPS) are two inflammatory diseases with very similar clinical manifestations. HPS is one of the most serious complications of AOSD and its risk of death is very high. It is difficult to identify HPS early in patients with AOSD, but early identification and proper treatment directly affects the prognosis.
A 39-year-old male showed a high spiking fever and myalgia. Laboratory data revealed elevated white blood cell, serum ferritin, and neutrophil percentage. However, his fever failed to relieve after a clear diagnosis of AOSD caused by pulmonary infection and treatment by antibiotics and corticosteroids; further laboratory data showed elevated serum ferritin, C-reactive protein, erythrocyte sedimentation rate and triglyceride, as well as liver abnormalities. Bone marrow smear showed hemophagocytosis. Secondary HPS was definitely diagnosed. The high fever disappeared and the laboratory findings returned to normal values after treatment by high-dose intravenous methylprednisolone and methotrexate.
For AOSD patients with high suspicion of HPS, active examination needs to be considered for early diagnosis, and timely using of adequate amount of corticosteroids is the key to reducing risk of HPS death.
成人斯蒂尔病（AOSD）和噬血细胞综合征（HPS）是两种临床表现非常相似的炎症性疾病。HPS是AOSD最严重的并发症之一，其死亡风险非常高。在AOSD患者中早期识别HPS很困难，但早期识别和恰当治疗直接影响预后。
一名39岁男性表现为高热和肌痛。实验室检查数据显示白细胞、血清铁蛋白和中性粒细胞百分比升高。然而，在明确诊断为肺部感染所致AOSD并经抗生素和糖皮质激素治疗后，其发热仍未缓解；进一步实验室检查数据显示血清铁蛋白、C反应蛋白、红细胞沉降率和甘油三酯升高，以及肝脏异常。骨髓涂片显示噬血细胞现象。确诊为继发性HPS。经大剂量静脉注射甲泼尼龙和甲氨蝶呤治疗后，高热消失，实验室检查结果恢复正常。
对于高度怀疑HPS的AOSD患者，需考虑积极检查以早期诊断，及时使用足量糖皮质激素是降低HPS死亡风险的关键。
