非霍奇金淋巴瘤合并噬血细胞综合征的临床与预后分析

Clinical and prognostic analyses of non-Hodgkin lymphoma combined with hemophagocytic syndrome

目的·比较非霍奇金淋巴瘤合并噬血细胞综合征(HPS)与不合并HPS患者的临床特征和预后差异。方法·对2006年1月—2014年5月住院治疗的非霍奇金淋巴瘤合并HPS患者33例(合并HPS组)及同时期未合并HPS的非霍奇金淋巴瘤患者76例(对照组)进行回顾性分析。采用Kaplan-Meier法进行生存分析,利用Cox回归模型对可能影响生存的因素进行单因素和多因素分析。结果·合并HPS组多以发热起病,大部分骨髓样本(29/33)中可以找到噬血细胞,血清铁蛋白、三酰甘油、总胆红素和乳酸脱氢酶(LDH)明显升高,分期晚、体能状态差、病情重,总体预后较对照组差(P=0.000)。合并HPS及治疗未达完全缓解是非霍奇金淋巴瘤患者预后差的独立危险因素。结论·非霍奇金淋巴瘤合并HPS的患者病情重,总体预后较差。

Objective · To compare the clinical features and prognosis between non-Hodgkin lymphoma （NHL） patients with and without hemophagocytic syndrome （HPS）. Methods · A retrospective analysis was performed for 33 NHL patients with HPS （the HPS group） and 76 NHL patients without HPS （the control group） admitted from January 2006 to May 2014. Survival analysis was conducted with Kaplan-Meier method and univariate and multivariate analyses were carried out by using a COX regression model for factors that might affect the survival of patients. Results · The NHL patients with HPS were likely to start with fever. Most bone marrow samples （29/33） had elevated serum ferritin, triglyceride, total bilirubin, and lactate dehydrogenase （LDH） and hemophagocytes could be found in these samples. The NHL patients with HPS had late disease stage, poor physical ability, and severe disease status and their prognosis was poorer compared with controls （P=0.000）. Failure to achieve complete remission and the combination of HPS were independent risk factors for poor prognosis of NHL patients. Conclusion · The NHL patients with HPS have severe disease status and poor prognosis.