CAJ | 학술논문

目的：探讨成人噬血细胞性淋巴组织细胞增生症（HLH）的临床特点。方法：对2005年1月－2015年1月在南京医科大学第二附属医院血液科收治的21例成人 HLH 患者的临床资料进行回顾性分析。结果：21例患者初治时均表现为高热、肝脾肿大、凝血功能异常、血常规三系或二系受累。病因分析中7例（33．3％）为血液肿瘤相关性；10例（47．6％）为感染，其中3例确诊为发热伴血小板减少综合征（新型布尼亚病毒核酸检测阳性）；1例（4．8％）肿瘤相关性（骨髓活检示转移癌）；3例（14．3％）原因不明。随访中死亡16例，存活3例（最长至今已5年7个月），2例失访；生存时间为4天～5．7年（中位生存时间183天）。结论：HLH 为罕见的致死性疾病，成人更为少见。临床表现复杂，常伴有多脏器受损，病情凶险，进展迅速。预后大多不良。发病机制和治疗手段有待进一步研究。
목적：탐토성인서혈세포성림파조직세포증생증（HLH）적림상특점。방법：대2005년1월－2015년1월재남경의과대학제이부속의원혈액과수치적21례성인 HLH 환자적림상자료진행회고성분석。결과：21례환자초치시균표현위고열、간비종대、응혈공능이상、혈상규삼계혹이계수루。병인분석중7례（33．3％）위혈액종류상관성；10례（47．6％）위감염，기중3례학진위발열반혈소판감소종합정（신형포니아병독핵산검측양성）；1례（4．8％）종류상관성（골수활검시전이암）；3례（14．3％）원인불명。수방중사망16례，존활3례（최장지금이5년7개월），2례실방；생존시간위4천～5．7년（중위생존시간183천）。결론：HLH 위한견적치사성질병，성인경위소견。림상표현복잡，상반유다장기수손，병정흉험，진전신속。예후대다불량。발병궤제화치료수단유대진일보연구。
Objective:To investigate the clinical characteristics of adult patients with hemophagocytic lymphohisto-cytosis(HLH).Methods:The clinical and experimental data of 21 adult patients with HLH from Department of He-matology,The Second Affiliated Hospital of Nanjing Medical University,were collected retrospectively from January 2005 to January 2015.Results:All patients presented high fever,hepatosplenomegaly,hematostatic abnormality,and cytopenia at first visit.Seven cases(33.3%)were diagnosed as non -Hodgkins'lymphoma.10(47.6%)were diag-nosed as infection,3 cases as fever with thrombocytopenia syndrome among them.1(4.8%)was malignancy -associ-ated HLH,3(14.3%)without pathogenesis.Seventeen cases were dead during follow -up and two was still in follow-up(the longest was 5 years and 7 months till now).Two cases lost follow -up.The median survival time was 183 days(range from 4 days ~5.7 years).Conclusion:HLH is an uncommon fatal disease and farely occurred in adults. The clinical presentation is complex,usually with multi -organ dysfunction,aggressive course and poor prognosis.The pathogenesis and treatment of HLH should be further studied.