Department of Internal Medicine, Tainan Municipal Hospital, Tainan, Taiwan.
J Formos Med Assoc. 2010 Jan;109(1):85-8. doi: 10.1016/s0929-6646(10)60026-5.
Hemophagocytic syndrome (HS) that occurs in the course of adult-onset Stills disease (AOSD) has been reported only rarely in the literature. HS and AOSD share overlapping clinical and laboratory features, therefore, it is difficult to recognize HS as a complication of AOSD. Here, we report the case of a 46-year old woman with classical features of AOSD. Severe pancytopenia and jaundice associated with extreme hyperferritinemia occurred during high-dose steroid treatment. Bone marrow biopsy showed typical pathological features of hemophagocytosis, which confirmed the coexistence of HS with AOSD. The patient was treated with methylprednisolone pulse therapy of 500 mg/day for 3 days, as recommended in cases of HS complicating AOSD, and her condition improved gradually. During the disease course, extensive studies could not identify any viral infection or other known underlying etiology for the reactive hemophagocytosis. Currently, the patient is in remission on low-dose prednisolone and azathioprine.
噬血细胞综合征（HS）在成人Still 病（AOSD）病程中发生的情况在文献中仅少有报道。HS 和 AOSD 具有重叠的临床和实验室特征，因此，很难将 HS 识别为 AOSD 的并发症。在这里，我们报告了一例 46 岁女性，具有典型的 AOSD 特征。在大剂量类固醇治疗期间，出现严重的全血细胞减少和黄疸，伴有极高的铁蛋白血症。骨髓活检显示噬血细胞的典型病理特征，证实了 HS 与 AOSD 并存。根据 HS 合并 AOSD 的建议，该患者接受了 500mg/天的甲基强的松龙脉冲治疗 3 天，病情逐渐改善。在疾病过程中，广泛的研究未能确定任何病毒感染或其他已知的潜在病因引起的反应性噬血细胞。目前，该患者正在接受低剂量泼尼松龙和硫唑嘌呤治疗，处于缓解期。
