成人史提爾氏病合併嗜血症候群：一個病例報告與文獻回顧

Adult-onset Still's Disease Associated with Hemophagocytic Syndrome: A Case Report and Literature Review

報告台灣第一個成人史提爾氏病合併嗜血症候群的病例。患者是24歲男性因不明熱住院，最後診斷為成人史提爾氏病，其骨髓檢查出現嗜血細胞過多的情況，在排除包括感染與其他可能引起反應性嗜血症候群的原因後，確認其嗜血症候群與成人史提爾氏病有關。患者在非類固醇抗發炎藥物與類由醇的治療下康復。我們回顧文獻中成人史提爾氏病合併嗜血症候群的病例，嗜血症候群多發生在成人史提爾氏病發病的時侯，且其中大約有一半的病患的白血球增加或正常。這與本病例的情況相同。我們並討論可能的病理機轉，兩者的相關性與治療方針。

We described the first case of hemophagocytosis in adult onset Still's disease (AOSD) in Taiwan. The 24-year-old male was admitted to our hospital for fever of unknown origin. The diagnosis of AOSD was made according to the 1992 criteria, proposed by Yamaguchi. Extensive studies could not identify any infection or other known underlying disorders for reactive hemophagocytosis except for active AOSD. The patient responded well to non-steroidal anti-inflammatory drugs and glucocorticoid. Hemophagocytic syndrome (HPS) often occurred at the onset of AOSD and half of them did not have leukopenia in the literature reviewed. Our case had the same condition. Pathogenetic mechanism and possible association between AOSD and reactive HPS, and the treatment of HPS associated with AOSD are also discussed.