重症成人Still病1例报道并文献复习  

Severe adult onset Still＇s disease： a case report and literature review

作　　者：王敏[1] 郑瑶[1] 杨旻[1] Wang Min;Zheng Yao;Yang Min(Department of Intensive Care Medicine,the Second Affiliated Hospital of Anhui Medical University,Hefei 230032,China.Department of Cardiopulmonary Resuscitation and Critical Care Laboratory,the Second Affiliated Hospital of Anhui Medical University,Hefei 230023,Chin)

机构地区：[1]安徽医科大学第二附属医院重症医学科安徽医科大学第二附属医院心肺复苏与危重病研究室,安徽合肥230032

出　　处：《中国急救医学》2018年第8期700-705,共6页Chinese Journal of Critical Care Medicine

基　　金：国家自然科学青年研究基金（81601661）;安徽省自然科学基金面上项目（1608085MH195）

摘　　要：目的 探讨成人Still病（adult onset Still′s disease，AOSD）继发噬血细胞综合征（hemophagocytic syndrome，HPS）的临床资料及预后。 方法 以“adult onset Still′s disease”、“macrophage activation syndrome”、“hemophagocytic lymphohistiocytosis” 和 “hemophagocytic syndrome”为检索词检索PubMed数据库，以“（成人斯蒂尔病 or 成人 Still′s病）、（巨噬细胞活化综合征 or 嗜血细胞综合征 or 噬血细胞综合征）”为检索词检索CNKI、WangFang Data和VIP数据库，时限为建库~2016年。 结果 患者，女，34岁，发热伴皮疹、咽痛、关节痛半个月,于2017-10-04入院。初步诊断发热待查，予以多种抗菌药物治疗后体温持续8 d未见下降，甲强龙使用48 h后体温再次上升，且出现血压下降、呼吸困难，骨穿见嗜血细胞，诊断AOSD、HPS、多脏器功能障碍综合征（MODS）。经地塞米松、环孢素（cyclosporine A, CsA）、依托泊苷（etoposide, VP16）治疗好转。检索文献54篇，其中5例因数据不全被排除，余下的49例，女39例，男10例，平均年龄（34.8±13.4）岁。HPS临床表现中发热、脾大、吞噬现象发生率高，血小板减少、铁蛋白、肝酶增高显著。HPS患者激素用量多，CsA、丙种球蛋白（IVIG）使用比例高，4例接受VP16治疗。11例死亡，5例死于MODS，3例死于中枢神经系统感染，余3例分别死于肠出血、呼吸衰竭、间质性心肌炎。 结论 HPS诊治关键在于早期诊断；早期激素联合免疫抑制剂治疗AOSD继发HPS（如出现持续血三系下降，积极应用VP16），合并MODS预后差。Objective To investigate the clinical characteristic of hemophagocytic syndrome （HPS） secondary to adult onset Still＇s disease （AOSD）. Methods We searched database including PubMed, CNKI, Wang Fang Data and VIP from inception to 2016, to collect reports for patients with liPS or macrophage activation syndrome or hemophagocytic lymphohistiocytosis secondary to AOSD. Meanwhile, literature was reviewed. Results A female patient, 34 years old, presented with a halfmonth history of spiking fever, associated with pharyngalgia, arthralgia and an evanescent rash and hospitalized in October 2017. After admission, the patient was diagnosed as fever of unknown origin. The body temperature was not decreased after 8 days of treatment with broad - spectrum antibiotics and nonsteroidal anti -inflammatory drugs （NSAID）. The body temperature increased again within 48 hours after administration of glucocorticoid, and her condition suddenly became deteriorated and developed hypotension, progressive dyspnea on 10 days of institution. Bone marrow biopsy revealed hemophagocytosis. She was diagnosed as HPS secondary to AOSD. The patient was treated with dexamethasone and cyclosporine A （CsA）. Intravenous immunogloblin （IVIG） and topoisomerase II inhibitor etoposide （VP16） were also applied. The patient was improved. After exclusion those without full medical history and complete data 49 cases in detailed reports were enrolled, including 39 females and 10 males. The mean age was （34.8 ＋ 13.4） years. Clinical manifestations of HPS secondary to AOSD were recurrently fever, lymphadenopathy and hepatosplenomegaly, and the characteristic of laboratory test are cytopenia, the increasingly serum ferritin （SF）, liver injury and phagocytois found in bone marrow biopsy at the early stage of cytopenia. Glucocorticoids were used in 47 patients. The percentage of patients received IVIG and CsA were significantly higher than those who receiving VP16. Eleven patients died, 3 of whom died of central nervous system in

关 键 词：成人Still病(AOSD) 嗜血细胞活化综合征(HPS) 多脏器功能障碍综合征(MODS) 依托泊苷(VP16) 

分 类 号：R593.22[医药卫生—内科学]