成年人继发性噬血细胞综合征临床特点及预后分析

Clinical characteristics and prognostic analysis of secondary hemophagocytic syndrome in adults

目的探讨成年人继发性噬血细胞综合征(HPS)的临床特点及预后。方法回顾性分析2014年1月至2020年6月四川省绵阳市中心医院收治的43例初发继发性HPS成年患者的临床资料。总结患者临床表现及生化指标。比较铁蛋白高水平组(≥5 000 μg/L)和低水平组(<5 000 μg/L)间、淋巴瘤相关HPS组与感染相关HPS组间患者的临床特点。采用logistic回归模型进行多因素生存分析。结果患者均以发热为主诉,起病时或治疗中均有感染表现。88.4%(38/43)患者骨髓有噬血现象;88.4%(38/43)患者有不同程度的血细胞减少;36.6%(15/41)患者纤维蛋白原降低[中位值0.93 g/L(0.50~1.35 g/L)];76.7%(33/43)患者氨基转移酶升高。与铁蛋白低水平组(19例)相比,高水平组(21例)单核细胞比例低(0.081±0.040比0.163±0.076,P=0.016),纤维蛋白原降低发生率及氨基转移酶升高发生率均高[60.0%(12/20)比11.1%(2/18),P=0.002;95.2%(20/21)比52.6%(10/19),P=0.006]。与感染相关HPS患者相比,淋巴瘤相关HPS患者淋巴结大发生率高[72.7%(16/22)比27.8% (5/18),χ2=8.021,P=0.005],氨基转移酶升高发生率低[59.1%(13/22)比94.4%(17/18),χ2=4.848,P=0.028]。多因素分析显示,治疗后中性粒细胞比例高(OR=0.886,95%CI 0.786~0.998,P=0.046)及清蛋白水平高(OR=0.376,95%CI 0.156~0.907,P=0.030)是患者生存的保护性因素。结论初发继发性HPS成年患者中,铁蛋白≥5 000 μg/L者单核细胞比例降低,更易出现肝损害及纤维蛋白原降低。淋巴瘤相关HPS患者更易出现淋巴结大,感染相关HPS患者更易出现肝损害。治疗后中性粒细胞比例低、低蛋白血症提示继发性HPS成年患者预后较差。

Objective To investigate the clinical characteristics and prognosis of secondary hemophagocytic syndrome(HPS)in adults.Methods The clinical data of 43 adult patients with primary secondary HPS in Mianyang Central Hospital of Sichuan Province from January 2014 to June 2020 were retrospectively analyzed.The patient's clinical manifestations and biochemical indicators were summarized.The clinical characteristics of patients between high-level ferritin(≥5000μg/L)group and low-level ferritin(<5000μg/L)group,lymphoma-related HPS group and infection-related HPS group were compared.Logistic regression model was used for multivariate survival analysis.Results All patients had fever as the main complaint,and infections were manifested at the onset or during treatment.In all patients,88.4%(38/43)had hemophagocytosis in the bone marrow;88.4%(38/43)had varying degrees of cytopenia;36.6%(15/41)had reduced fibrinogen[median value 0.93 g/L(0.50-1.35 g/L)];76.7%(33/43)had elevated aminotransferase.Compared with the low-level ferritin group(19 cases),the high-level group(21 cases)had a lower proportion of monocytes(0.081±0.040 vs.0.163±0.076,P=0.016),the incidence of fibrinogen reduction and the incidence of aminotransferase elevation were high[60.0%(12/20)vs.11.1%(2/18),P=0.002;95.2%(20/21)vs.52.6%(10/19),P=0.006].Compared with infection-related HPS patients,lymphoma-related HPS patients had a higher incidence of lymphadenopathy[72.7%(16/22)vs.27.8%(5/18),χ2=8.021,P=0.005]and a lower incidence of aminotransferase elevation[59.1%(13/22)vs.94.4%(17/18),χ2=4.848,P=0.028].Multivariate analysis showed that the high proportion of neutrophils(OR=0.886,95%CI 0.786-0.998,P=0.046)and high-level albumin(OR=0.376,95%CI 0.156-0.907,P=0.030)after treatment were protective factors for survival of patients.Conclusions Among adult patients with primary secondary HPS,the proportion of monocytes in patients with ferritin≥5000μg/L is low,and liver damage and fibrinogen reduction are more likely to occur.Lymphoma-related HPS patients are more likely to have lymphadenopathy,and infection-related HPS patients are more likely to have liver damage.After treatment,a low proportion of neutrophils and hypoproteinemia indicate a poor prognosis in adult patients with secondary HPS.