151例初诊继发性噬血细胞性淋巴组织细胞增多症患者的临床特征及预后因素分析  

Analysis of clinical characteristics and prognostic factors of 151newly diagnosed patients with secondary hemophagocytic lymphohistiocytosis

作　　者：高欣 段丽敏 许戟 田甜 王菊娟 尹光丽 仇红霞 GAO Xin;DUAN Limin;XU Ji;TIAN Tian;WANG Jujuan;YIN Guangli;QIU Hongxia(Department of Hematology,First Affiliated Hospital,Nanjing Medical University,Nanjing 210029,CHINA)

机构地区：[1]南京医科大学第一附属医院血液科,江苏210029

出　　处：《江苏医药》2024年第12期1198-1203,共6页Jiangsu Medical Journal

基　　金：国家自然科学基金项目(81570175)。

摘　　要：目的探讨继发性噬血细胞性淋巴组织细胞增多症(sHLH)患者的临床特点和预后因素。方法回顾性分析151例初诊sHLH患者的临床特征及相关预后影响因素。结果不同病因sHLH各组间性别构成比、脾肿大、血清钙离子和血清铁蛋白水平差异有统计学意义(P<0.05)。151例sHLH患者1年、2年和3年总生存率分别为63.2%、52.2%和47.5%。肿瘤相关、感染相关、自身免疫性疾病相关和不明原因sHLH患者的1年生存率分别为62.1%、51.3%、85.7%和80.4%。单因素Cox回归分析结果显示,低血小板计数、低纤维蛋白原血症、低血清钙离子水平和高三酰甘油水平是初诊sHLH患者死亡的危险因素(P<0.05);多因素Cox回归分析结果显示,低血小板计数、低纤维蛋白原血症和低血清钙离子水平是初诊sHLH患者死亡的独立危险因素(P<0.05)。结论动态监测sHLH患者的血小板计数、纤维蛋白原水平、血清钙离子水平和三酰甘油水平有助于评估病情变化和治疗反应,有利于及时调整治疗方案。Objective To investigate the clinical characteristics and related prognostic factors of patients with secondary hemophagocytic lymphohistiocytosis(sHLH).Methods The clinical characteristics and relevant factors influencing the prognosis were retrospectively analyzed in 151 patients with newly diagnosed sHLH.Results The differences in gender distribution,splenomegaly,serum calcium concentration and serum ferritin among the patient groups with different etiologies of sHLH were statistically significant(P<0.05).The overall survival rates of the 151 sHLH patients newly diagnosed at 1,2 and 3 years were 63.2%,52.2%and 47.5%,respectively.The 1-year survival rates for tumor-associated,infection-associated,autoimmune disease-associated and unknown cause sHLH were 62.1%,51.3%,85.7%and 80.4%,respectively.The results of univariate Cox regression analysis showed that low platelet count,hypofibrinogenemia,low serum calcium concentration and high triglyceride level were the risk factors for death in newly diagnosed sHLH patients(P<0.05).Multivariate Cox regression analysis showed that low platelet count,hypofibrinogenemia and low serum calcium concentration were the independent risk factors for death in newly diagnosed sHLH patients(P<0.05).Conclusion Dynamic monitoring of platelet count,fibrinogen level,serum calcium concentration and triglyceride level in the patients with sHLH can help to evaluate the changes of the disease and response to treatment,which is helpful in timely adjusting the treatment.

关 键 词：噬血细胞性淋巴组织细胞增多症 

分 类 号：R733[医药卫生—肿瘤]