白血病·淋巴瘤
白血病·淋巴瘤
백혈병·림파류
JOURNAL OF LEUKEMIA & LYMPHOMA
2014年
9期
541-545
,共5页
曾祥宗%王旖旎%王晶石%吴林%张嘉%马骥良%黄文秋%陈建行%裴瑞君
曾祥宗%王旖旎%王晶石%吳林%張嘉%馬驥良%黃文鞦%陳建行%裴瑞君
증상종%왕의니%왕정석%오림%장가%마기량%황문추%진건행%배서군
噬血细胞综合征%自身免疫病%临床表现%实验室指标%治疗%预后
噬血細胞綜閤徵%自身免疫病%臨床錶現%實驗室指標%治療%預後
서혈세포종합정%자신면역병%림상표현%실험실지표%치료%예후
Hemophagocytic syndromes%Autoimmune diseases%Clinical features%Laboratory examination%Treatment%Prognosis
目的 提高对自身免疫病相关噬血细胞综合征(AAHS)的认识和诊疗水平.方法 对2004年4月至2014年4月间确诊为AAHS患者的临床表现、实验室检查、诊断、治疗方案及临床转归进行回顾性综合分析.结果 29例AAHS患者中女22例,男7例,中位年龄31岁(16~70岁).原发病为成年人斯蒂尔病17例,干燥综合征5例,系统性红斑狼疮4例,多发性肌炎1例,原发性胆汁性肝硬化1例,未分化结缔组织病1例.19例患者在自身免疫病(AID)治疗过程中继发噬血细胞综合征(HPS),10例患者AID与HPS同时发生.AAHS的主要临床表现以发热(100.0%,29/29)、呼吸系统症状(79.3%,23/29)、脾大(72.4%,21/29)、皮疹(65.5%,19/29)、关节疼痛(62.1%,18/29)最为常见.29例AAHS患者治疗8周内,13例临床有效,8例达到临床缓解.患者1、3、12个月总生存率分别为93.1%、82.3%、72.0%.结论 AAHS患者的原发病以成年人斯蒂尔病、干燥综合征、系统性红斑狼疮为主,HPS与AID可同时发生,也可以在AID治疗过程中继发.当HPS患者伴有皮疹及关节疼痛时需考虑到AID的可能.与其他类型的HPS患者相比,AAHS的预后相对较好.
目的 提高對自身免疫病相關噬血細胞綜閤徵(AAHS)的認識和診療水平.方法 對2004年4月至2014年4月間確診為AAHS患者的臨床錶現、實驗室檢查、診斷、治療方案及臨床轉歸進行迴顧性綜閤分析.結果 29例AAHS患者中女22例,男7例,中位年齡31歲(16~70歲).原髮病為成年人斯蒂爾病17例,榦燥綜閤徵5例,繫統性紅斑狼瘡4例,多髮性肌炎1例,原髮性膽汁性肝硬化1例,未分化結締組織病1例.19例患者在自身免疫病(AID)治療過程中繼髮噬血細胞綜閤徵(HPS),10例患者AID與HPS同時髮生.AAHS的主要臨床錶現以髮熱(100.0%,29/29)、呼吸繫統癥狀(79.3%,23/29)、脾大(72.4%,21/29)、皮疹(65.5%,19/29)、關節疼痛(62.1%,18/29)最為常見.29例AAHS患者治療8週內,13例臨床有效,8例達到臨床緩解.患者1、3、12箇月總生存率分彆為93.1%、82.3%、72.0%.結論 AAHS患者的原髮病以成年人斯蒂爾病、榦燥綜閤徵、繫統性紅斑狼瘡為主,HPS與AID可同時髮生,也可以在AID治療過程中繼髮.噹HPS患者伴有皮疹及關節疼痛時需攷慮到AID的可能.與其他類型的HPS患者相比,AAHS的預後相對較好.
목적 제고대자신면역병상관서혈세포종합정(AAHS)적인식화진료수평.방법 대2004년4월지2014년4월간학진위AAHS환자적림상표현、실험실검사、진단、치료방안급림상전귀진행회고성종합분석.결과 29례AAHS환자중녀22례,남7례,중위년령31세(16~70세).원발병위성년인사체이병17례,간조종합정5례,계통성홍반랑창4례,다발성기염1례,원발성담즙성간경화1례,미분화결체조직병1례.19례환자재자신면역병(AID)치료과정중계발서혈세포종합정(HPS),10례환자AID여HPS동시발생.AAHS적주요림상표현이발열(100.0%,29/29)、호흡계통증상(79.3%,23/29)、비대(72.4%,21/29)、피진(65.5%,19/29)、관절동통(62.1%,18/29)최위상견.29례AAHS환자치료8주내,13례림상유효,8례체도림상완해.환자1、3、12개월총생존솔분별위93.1%、82.3%、72.0%.결론 AAHS환자적원발병이성년인사체이병、간조종합정、계통성홍반랑창위주,HPS여AID가동시발생,야가이재AID치료과정중계발.당HPS환자반유피진급관절동통시수고필도AID적가능.여기타류형적HPS환자상비,AAHS적예후상대교호.
Objective To analyze the clinical characters,treatment and prognosis of autoimmune disease-associated hemophagocytic syndromes (AAHS).Methods A retrospective study was carried out to analyze the clinical features,laboratory tests,diagnosis,treatments and clinical outcomes of 29 patients with AAHS from Apr.2004 to Apr.2014.Results Among the 29 cases,22 were female while the other 7 were male.The median age was 31 years old (16-70 years old).The initiating diseases consisted of 17 adult onset still's disease,5 sicca syndrome,4 systemic lupus erythematosus,1 polymyositis,primary,1 biliary cirrhosis,1 undifferentiated connective tissue disease.19 patients of them were diagnosed as hemophagocytic syndromes (HPS) in the treatment of autoimmune disease while the other 10 patients were diagnosed as autoimmune disease and HPS at the same time.The most common clinical features were persistent fever (100.0 %,29/29),respiratory symptom (79.3 %,23/29),splenomegaly (72.4 %,21/29),rash (65.5 %,19/29),arthralgia (62.1%,28/29).Among the 29 patients,13 patients had acquired clinical effective and 8 patients had acquired clinical alleviate within 8 weeks treatment.Among the 29 cases,1-month,3-month and 1-year overall survival rates were 93.1%,82.3 % and 72.0 % respectively.Conclusions The initiating diseases of AAHS mainly consist of adult onset still's disease,sicca syndrome and systemic lupus erythematosus.HPS can not only onset at the same time with autoimmune disease,but also onset in the treatment of autoimmune disease.Autoimmune disease should be considered when HPS patients complicated by rash and arthralgia.Compared with other types of HPS patients,the prognosis of AAHS is better relatively.