摘要
目的:了解继发性噬血细胞综合征(HLH)的病因、临床特征及预后,以提高对HLH的认识,减少临床HLH的误诊和漏诊率。方法:收集2015年1月至2021年12月经本院收治的75例成人继发性HLH患者的病历资料,对其病因、临床特点、实验室检查结果、治疗及预后等进行回顾性分析。随访至末次出院时间。结果:75例患者中,感染相关HLH最常见(45.33%),其次为淋巴瘤相关HLH(17.33%)。临床表现以发热最为常见(97.67%),实验室指标中NK细胞活性(98.31%降低)、sCD25浓度(93.22%升高)、血清铁蛋白(94.44%升高)在诊断中灵敏度更高。将不同病因HLH患者初诊时临床表现及实验室指标进行比较,性别、淋巴结肿大、骨髓形态是否可见噬血现象对原发病的诊断更有价值(均P<0.05)。将不同病因HLH患者的治疗方案与临床转归进行比较,自身免疫性疾病相关HLH采用激素+环孢素治疗临床缓解率最高(83.3%)(P<0.05)。患者总体12个月的生存率为26.7%,其中感染相关HLH患者12个月的生存率最低(14.7%),自身免疫性疾病相关HLH患者12个月的生存率最高(63.6%)。结论:成人继发性HLH病因和临床特点多样,预后凶险,病情严重程度具有异质性,早期明确病因对HLH预后具有重要意义,需要进一步提高对HLH的认识。
Objective:To understand the etiology,clinical characteristics and prognosis of secondary hemophagocytic syndrome(HLH),so as to improve the understanding of HLH and reduce the rates of misdiagnosis and missed diagnosis of HLH.Methods:A retrospective study was conducted to analyze the cause,clinical characteristics,laboratory findings,therapy and outcomes of 75 adult patients with secondary HLH admitted to our hospital from January 2015 to December 2021.Follow-up continued until the last discharge time.Results:Among 75 patients,infection-related HLH was the most common(45.33%),followed by lymphoma-related HLH(17.33%).Fever was the most common clinical manifestation(97.67%).Laboratory indicators such as NK cell activity(98.31%low or absent),sCD25(93.22%increased),and serum ferritin(94.44%elevated)had higher sensitivity in diagnosis.By comparing the clinical manifestations and laboratory indicators of HLH patients with different causes,sex,lymph node enlargement and bone marrow morphology were more valuable for the diagnosis of primary disease(all P<0.05).By comparing the treatment and clinical outcomes of HLH patients with different causes,the highest clinical remission rate(83.3%)was achieved in patients with autoimmune disease-related HLH treated with hormone+cyclosporine(P<0.05).The overall 12-month survival rate of all patients was 26.7%,in which the infection-related HLH was the lowest(14.7%)while autoimmune disease-related HLH was the highest(63.6%).Conclusion:The causes and clinical characteristics of adult secondary HLH are varied,with poor prognosis and heterogeneity in disease severity.It is important to identify HLH cause early for diagnosis and needed to further understand HLH.
作者
张雅丽
郝静楠
孙萌萌
邢晓英
乔淑凯
ZHANG Ya-Li;HAO Jing-Nan;SUN Meng-Meng;XING Xiao-Ying;QIAO Shu-Kai(General Medical Depurtment,The Second Hospital of Hebei Medical University,Shijiczhucang 050000,Hebei Province,China;Department of Hematology,The Second Hospital of Hebei Medical University,Shijiczhucang 050000,Hebei Province,China)
出处
《中国实验血液学杂志》
CAS
CSCD
北大核心
2024年第4期1230-1237,共8页
Journal of Experimental Hematology
关键词
继发性噬血细胞综合征
病因
临床特点
预后
secondary hemophagocytic syndrome
pathogeny
clinical char acteristics
prognosis
