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摘要: 目的 研究成人继发性噬血细胞综合征(HLH)患者的临床特征及预后相关因素。方法 回顾性分析2019年9月—2021年2月就诊于我院血液科的95例初诊继发性HLH患者的临床资料，分析其临床特征、治疗及预后因素。结果 95例患者的中位年龄51(19~79)岁；最常见病因为淋巴瘤，共34例(35.8%)。95例患者中64例(67.4%)死亡，中位生存时间72 d。Cox单因素分析表明，成人继发性HLH的预后受性别、中性粒细胞水平、血小板计数、肌酐、尿素氮、活化部分凝血活酶时间等因素影响(P< 0.05)。多因素Cox回归分析结果发现，中性粒细胞 < 1.0×109/L、活化部分凝血活酶时间>50 s、尿素氮升高是成人继发性HLH的独立预后影响因素(P< 0.05)。结论 成人继发性HLH的预后受多方面因素影响，中性粒细胞 < 1.0×109/L、活化部分凝血活酶时间>50 s、尿素氮升高是患者死亡的独立危险因素。Abstract: Objective To explore the clinical features and prognostic factors of adult patients with secondary hemophagocytic syndrome(HLH).Methods The clinical data of 95 newly diagnosed patients with secondary HLH who were hospitalized from September 2019 to February 2021 were retrospectively analyzed, and clinical features, survival, and prognostic factors of patients were analyzed.Results The median age of 95 patients was 51(19-79) years old. The most common cause was lymphoma, with 34 cases(35.8%). Of the 95 patients, 64 cases(67.4%) died and the median survival time was 72 days. Cox univariate analysis showed that sex, neutrophil level, platelet count, creatinine, urea nitrogen, and activated partial thromboplastin time were related to the prognosis of secondary HLH in adults(P< 0.05). Multivariate Cox regression analysis showed that neutrophil < 1.0×109/L, activated partial thromboplastin time>50 s, and elevated urea nitrogen were independent prognostic factors for secondary HLH in adults(P< 0.05).Conclusion The prognosis of adult secondary HLH is affected by many factors, neutrophil < 1.0×109L, activated partial thromboplastin time>50 s and elevated urea nitrogen are independent risk factors of death.
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Key words:
- adult /
- hemophagocytic syndrome /
- clinical features /
- prognostic analysis
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表 1 生存组与死亡组患者间发病年龄及检查结果比较
M(P25，P75) 因素 存活组(64例) 死亡组(31例) Z P 发病年龄/岁 48.0(32.0，58.0) 53.0(39.5，65.0) -1.485 0.138 血红蛋白/(g·L-1) 81.0(69.0，95.0) 83.0(70.3，93.0) -0.048 0.962 中性粒细胞/(×109·L-1) 3.9(1.1，5.3) 1.3(0.7，2.9) -0.279 0.005 PLT/(×109·L-1) 50.0(25.0，91.0) 22.5(12.3，41.8) -3.593 0.001 CRP/(mg·L-1) 64.0(31.6，112.9) 49.4(20.3，93.7) -0.633 0.527 降钙素原/(ng·mL-1) 1.0(0.4，5.1) 0.5(0.2，1.4) -2.029 0.042 ALT/(U·L-1) 93.0(35.0，128.0) 68.0(37.8，208.8) -0.127 0.899 AST/(U·L-1) 113.0(49.0，334.0) 144.5(52.0，340.0) -0.452 0.651 LDH/(U·L-1) 828.5(482.3，870.5) 1 255.0(574.0，2 469.0) -1.458 0.145 纤维蛋白原/(g·L-1) 1.3(0.9，3.0) 1.3(1.0，2.1) -0.131 0.896 TT/s 19.5(17.6，26.4) 20.6(17.7，28.0) -0.548 0.584 PT/s 13.2(12.4，14.5) 12.8(11.7，14.7) -0.619 0.536 APTT/s 31.9(29.4，38.3) 36.2(30.7，48.3) -2.084 0.037 D-二聚体/(mg·L-1) 6.8(2.3，17.1) 3.5(1.0，10.0) -1.631 0.103 肌酐/(μmol·L-1) 50.0(40.0，63.0) 67.0(49.5，86.0) -2.747 0.006 尿素氮/(mmol·L-1) 4.7(4.1，6.1) 7.4(5.3，10.1) -3.667 0.001 总胆红素/(μmol·L-1) 14.5(8.6，28.6) 23.4(13.5，61.3) -2.155 0.031 白蛋白/(g·L-1) 26.1(24.6，31.2) 26.7(23.6，29.1) -0.329 0.742 血钙/(mmol·L-1) 2.0(1.9，2.1) 2.0(1.9，2.1) -1.142 0.253 血钠/(mmol·L-1) 135.0(132.0，138.0) 135.0(131.0，138.0) -1.079 0.280 铁蛋白/(μmol·L-1) 15 043.5(1 962.3，41 067.3) 13 595.0(3 029.5，28 467.8) -0.219 0.827 甘油三酯/(mmol·L-1) 3.2(1.8，4.1) 2.7(2.1，3.6) -0.503 0.615 
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表 2 95例HLH患者预后影响因素的单因素和多因素分析
因素 单因素分析 多因素分析 HR(95%CI) P HR(95%CI) P 性别(男) 1.734(1.056~2.849) 0.030 1.176(0.671~2.061) 0.571 中性粒细胞 < 1.0×109/L 1.919(1.158~3.182) 0.013 1.723(1.027~2.894) 0.039 PLT < 100×109/L 3.332(1.044~10.635) 0.042 2.528(0.764~8.371) 0.129 肌酐 > 115 μmol/L 2.486(1.321~4.680) 0.005 1.133(0.512~2.504) 0.758 尿素氮 > 8.2 mmol/L 2.214(1.327~3.694) 0.002 1.897(1.065~3.381) 0.030 APTT > 50 s 2.261(1.245~4.108) 0.007 2.142(1.090~2.142) 0.027 降钙素原 > 4.6 ng/mL 0.523(0.225~1.215) 0.132 总胆红素 > 25 μmol/L 1.946(1.188~3.188) 0.080
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