CAJ | 학술논문

目的通过分析总结噬血细胞综合征(HPS)患者的临床及实验室特点,提高对该病的认识.方法对象为北京大学人民医院2007至2014年收治的77例HPS患者,对77例患者的病因、临床表现、实验室特征、治疗及预后等进行分析.结果 77例患者男女比例相当,诊断HPS时年龄3个月～85岁.原发HPS 5例.继发HPS 72例,其中单纯感染28例,血液系统肿瘤25例,自身免疫病11例,另有8例病因未明.近一半HPS患者与自身免疫病或血液系统肿瘤同时诊断.临床上以高热(100％)、肝脾(81.8％)及淋巴结肿大(40.3％)为主要表现.实验室检查方面,以血细胞减少(94.8％)、铁蛋白升高(93.2％)为主,纤维蛋白原减低占61.8％,其中6例合并弥漫性血管内凝血(DIC).高甘油三酯血症占一半以上.78.1％患者可在骨髓检查中见噬血细胞现象.自然杀伤细胞活性减低或缺失占95.2％,可溶性CD25抗原(sCD)升高所占比例为100％.其他脏器损害以肺部感染(36.4％)及肝损伤(33.3％)多见.约70％患者应用激素联合依托泊苷和(或)环孢素A治疗.8例自身免疫病患者应用激素冲击治疗(甲泼尼龙200 ～ 500 mg/d).53例病情好转出院,14例死亡,10例因病情加重放弃治疗出院.此预后不良的24例中5例合并DIC,5例合并多脏器衰竭.血液系统肿瘤病死率最高(52.0％),此后依次为单纯感染(25.0％)、自身免疫病(18.2％).结论自身免疫病和血液系统肿瘤在起病同时即可出现HPS表现,对可疑患者及时行相关实验室检查有助于早期明确诊断.合并DIC、多脏器受损的患者预后不良.
목적 통과분석총결서혈세포종합정(HPS)환자적림상급실험실특점,제고대해병적인식.방법 대상위북경대학인민의원2007지2014년수치적77례HPS환자,대77례환자적병인、림상표현、실험실특정、치료급예후등진행분석.결과 77례환자남녀비례상당,진단HPS시년령3개월～85세.원발HPS 5례.계발HPS 72례,기중단순감염28례,혈액계통종류25례,자신면역병11례,령유8례병인미명.근일반HPS환자여자신면역병혹혈액계통종류동시진단.림상상이고열(100％)、간비(81.8％)급림파결종대(40.3％)위주요표현.실험실검사방면,이혈세포감소(94.8％)、철단백승고(93.2％)위주,섬유단백원감저점61.8％,기중6례합병미만성혈관내응혈(DIC).고감유삼지혈증점일반이상.78.1％환자가재골수검사중견서혈세포현상.자연살상세포활성감저혹결실점95.2％,가용성CD25항원(sCD)승고소점비례위100％.기타장기손해이폐부감염(36.4％)급간손상(33.3％)다견.약70％환자응용격소연합의탁박감화(혹)배포소A치료.8례자신면역병환자응용격소충격치료(갑발니룡200 ～ 500 mg/d).53례병정호전출원,14례사망,10례인병정가중방기치료출원.차예후불량적24례중5례합병DIC,5례합병다장기쇠갈.혈액계통종류병사솔최고(52.0％),차후의차위단순감염(25.0％)、자신면역병(18.2％).결론 자신면역병화혈액계통종류재기병동시즉가출현HPS표현,대가의환자급시행상관실험실검사유조우조기명학진단.합병DIC、다장기수손적환자예후불량.
Objective To summarize the clinical features and laboratory data of 77 patients with hemophagocytic syndrome (HPS).Methods A total of 77 patients of HPS were continuously collected from 2007 to 2014 at our hospital.Their underlying diseases,clinical features,laboratory data,therapy and prognosis were analyzed.Results Their The patients aged from 3 months to 85 years.The gender ratio was roughly equal.Primary HPS was diagnosed in only 5 cases by gene detection Another 72 cases belonged to secondary HPS.The causes were infection (n =28),hematologic neoplasm (n =25),autoimmune diseases (AID,n =11) and unknown (n =8).HPS was the initial symptom in nearly half cases of hematologic neoplasm and AID.HPS was characterized by high fever (100％),hypersplenomegaly (81.8％) and lymphadenopathy (40.3％).Laboratory data showed cytopenia (94.8％),serum ferritin elevation (93.2％),hypofibrinogenemia (61.8％),hemophagocytosis in bone marrow (78.1％) and hypertriglyceridemia (55.3％).Low NK-cell activity (95.2％) and elevation of sCD25 (100％) were specific manifestations in HPS.Pulmonary infection (36.4％) and hepatic malfunction (33.3％) were common.Approximately 70％ were treated with HLH-2004.Pulse-dose corticosteroid therapy (methylprdnisolone 200-500 mg/d) was used in 8 AID patients.And 14 patients died and 10 withdrew treatment because of exacerbation.Five had complications of DIC and another 5 progressed into MODS.Neoplasm (52.0％) had the highest mortality in secondary HPS.And infection (25.0％) and AID (18.2％) followed.Conclusion Sometimes HPS occurs simultaneously with autoimmune disease or neoplasm.Relevant laboratory tests for suspected patients may aid an early diagnosis.Presence of DIC or MODS in HPS is possibly correlated with a poor prognosis and a high mortality.