CAJ | 학술논문

目的分析噬血细胞淋巴组织细胞增生症(HLH)的临床表现、实验室特点、治疗及预后.方法对2003至2013年北京友谊医院收治并确诊的192例资料完整的成人HLH患者的临床表现、实验室检查、诊断、治疗方案及临床转归等资料进行回顾性综合分析.结果 192例患者中肿瘤相关HLH 70例,感染相关HLH 64例,位居前列.HLH-2004诊断标准中各指标的符合率分别为:发热98.96％,铁蛋白升高94.27％,可溶性IL-2受体(sCD25)升高94.79％,NK细胞活性减低94.27％,两系或三系血细胞减少80.73％,脾大80.21％,噬血现象74.48％,低纤维蛋白原血症50.52％,高甘油三脂血症37.50％.此外,94.27％的患者存在肝功能异常,96.35％的患者存在轻度或重度感染,75.52％的患者存在凝血功能异常.出现中枢神经系统症状、皮疹的患者分别占19.27％、20.31％.比较肿瘤、感染、风湿免疫相关HLH三组患者初诊时各实验室指标,WBC、PLT、sCD25表达、ALT、AST、总胆红素、球蛋白(GLO)水平差异有统计学意义(P＜ 0.05).比较存活组和死亡组初诊时各实验室指标,WBC、PLT、白蛋白(ALB)、GLO、脑尿钠肽、肌酐、尿素氮水平两组差异有统计学意义(P＜0.05).结论继发性HLH与多种潜在疾病有关,成人以恶性肿瘤,尤其是T细胞淋巴瘤为主要诱因,其次是EB病毒感染.在HLH-2004的8条诊断标准中,发热、铁蛋白升高、NK细胞活性降低或缺如、sCD25升高灵敏性较高.风湿免疫相关的HLH患者其血细胞在初期可能不减低.WBC、PLT、ALB、GLO以及是否出现心功能不全、肾功能不全、凝血功能障碍可能是影响预后的指标.
목적 분석서혈세포림파조직세포증생증(HLH)적림상표현、실험실특점、치료급예후.방법 대2003지2013년북경우의의원수치병학진적192례자료완정적성인HLH환자적림상표현、실험실검사、진단、치료방안급림상전귀등자료진행회고성종합분석.결과 192례환자중종류상관HLH 70례,감염상관HLH 64례,위거전렬.HLH-2004진단표준중각지표적부합솔분별위:발열98.96％,철단백승고94.27％,가용성IL-2수체(sCD25)승고94.79％,NK세포활성감저94.27％,량계혹삼계혈세포감소80.73％,비대80.21％,서혈현상74.48％,저섬유단백원혈증50.52％,고감유삼지혈증37.50％.차외,94.27％적환자존재간공능이상,96.35％적환자존재경도혹중도감염,75.52％적환자존재응혈공능이상.출현중추신경계통증상、피진적환자분별점19.27％、20.31％.비교종류、감염、풍습면역상관HLH삼조환자초진시각실험실지표,WBC、PLT、sCD25표체、ALT、AST、총담홍소、구단백(GLO)수평차이유통계학의의(P＜ 0.05).비교존활조화사망조초진시각실험실지표,WBC、PLT、백단백(ALB)、GLO、뇌뇨납태、기항、뇨소담수평량조차이유통계학의의(P＜0.05).결론 계발성HLH여다충잠재질병유관,성인이악성종류,우기시T세포림파류위주요유인,기차시EB병독감염.재HLH-2004적8조진단표준중,발열、철단백승고、NK세포활성강저혹결여、sCD25승고령민성교고.풍습면역상관적HLH환자기혈세포재초기가능불감저.WBC、PLT、ALB、GLO이급시부출현심공능불전、신공능불전、응혈공능장애가능시영향예후적지표.
Objective To analyze the clinical manifestations,laboratory data,therapy,and prognosis in patients with hemophagocytic lymphohistiocytosis (HLH).Methods A retrospective study was carried out in 192 adult patients with HLH between 2003 and 2013.Results Of the 192 cases,70 cases were secondary to cancer and 64 cases secondary to infection.According to HLH-2004 criteria,the coincidence rate of indices were:fever (98.96％),high level of serum ferritin (94.27％),increased level of soluble interleukin-2 receptor (sCD25) (94.79％),decreased or absent activity of NK cells (94.27％),cytopenias (80.73％),splenomegaly (80.21％),hemophagocytosis in bone marrow,spleen or lymph nodes (74.48％),hypofibrinogenemia (50.52％),hypertriglyceridemia (37.50％).In addition,94.27％ of patients were presented with liver dysfunction,96.35％ with infections,and 75.52％ with coagulopathy.Incidences of central nervous system symptoms and rash were 19.27％ and 20.31％,respectively.Among cancer,infection and rheumatic group,there were statistically differences on white blood cells (WBC),platelet (PLT),sCD25,alanine aminotransferase,aspartate aminotransferase,total bilirubin and globulin (GLO) (P＜0.05).The differences of WBC,PLT,albumin (ALB),GLO,brain natriuretic peptide,creatinine,urea nitrogen between survival group and death group had statistical significance.Conclusions The secondary HLH occurs from various underlined diseases.Cancer,especially T-cell lymphoma,is the main cause,Secondly,it is EB virus infection.The diagnostic sensitive indicators are Persistent fever,higher level of serum ferritin,low or absent NK-cell activity,and increased sCD25 were the most valuable parameters for diagnosis.Cytopenias were not common in early phase of HLH secondary to rheumatic diseases.WBC,PLT,ALB,GLO could be used as the preliminary parameters for diagnosis.Cardiac insufficiency,renal insufficiency and coagulation dysfunction play important roles in prognosis.