伴噬血细胞综合征的T细胞淋巴瘤临床特点及生存分析

  目的　研究伴噬血细胞综合征（HPS）的T细胞淋巴瘤的临床特点及生存情况。方法　收集2006年1月至2011年12月上海交通大学医学院附属瑞金医院血液科就诊的30例伴HPS的T细胞淋巴瘤患者，以随机数字表法抽取的50例不伴HPS的T细胞淋巴瘤患者作对照，分析其临床特点、实验室检查及生存情况。结果　30例伴HPS的T细胞淋巴瘤组患者表现为高热100.00 %（30／30）、脾大96.67 %（29／30），两系以上血细胞减少93.33 %（28／30），骨髓噬血现象86.67 %（26／30），乳酸脱氢酶（LDH）升高100.00 %（30／30），高三酰甘油血症46.67 %（14／30），低纤维蛋白血症60.00 %（18／30），铁蛋白升高93.33 %（28／30）及肝损伤86.67 %（26／30），以上指标与50例不伴HPS的T细胞淋巴瘤患者相比较，差异均有统计学意义（χ2值分别为23.11、22.50、36.05、64.20、21.82、5.31、16.54、26.82、46.43，均 P＜0.05）。但浅表淋巴结肿大比例的发生率却不高，仅有33.33 %，这使对原发病的诊断造成比较大的困难。治疗上主要以CHOP方案或依托泊苷+地塞米松的联合化疗，虽然能使症状和实验检查指标得到改善，但中位数生存时间仅20 d。伴与不伴HPS的T细胞淋巴瘤患者生存曲线差异有统计学意义（χ2＝35.05，P＜0.0001）。结论　伴HPS的T细胞淋巴瘤，临床表现复杂，常伴有多脏器受损的表现，联合化疗可使疾病暂时缓解，但总的生存时间短，预后差，发病机制和治疗手段有待进一步研究。

Clinical features and prognostic analysis of T-cell lymphoma with hemophagocytic syndrome

Objective　To investigate the clinical characteristics and survival analysis of T-cell lymphoma with hemophagocytic syndrome (HPS). Methods　The clinical features, laboratory tests and survival of 30 patients of T-cell lymphoma with HPS were analyzed comparing with 50 random patients of T-cell lymphoma without HPS (control group), and the data were obtained from department of hematology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine from January 2006 to December 2011. Results　30 cases of T-cell lymphoma with HPS manifested for high fever [100.00 % (30/30)], splenomegaly [96.67 % (29/30)], more than two-line reduction in blood cells [93.33 % (28/30)], bone marrow with bloodthirsty phenomenon [86.67 % (26/30)], LDH increased [100.00 % (30/30)], hypertriglyceridemia [46.67 % (14/30)], low fibrin hyperlipidemia [60.00 % (18/30)], ferritin increased [93.33 % (28/30)], liver damaged [86.67 % (26/30)] from patient group, which were higher than those of control group, the differences were statistically significant (χ2 ＝ 23.11, 22.50, 36.05, 64.20, 21.82, 5.31, 16.54, 26.82, 46.43, all P < 0.05). But the incidence of superficial lymph nodes enlargement was not high, only 33.33 %, which made the diagnosis of primary disease difficult. Combined chemotherapy of CHOP or etoposide plus dexamethasone could make the symptoms and laboratory indicators improved, but the median survival time was only 20 days. Compared with the control group, there were significant differences on the survival curves (χ2 ＝ 35.05, P < 0.0001). Conclusion　In T-cell lymphoma with HPS, the clinical manifestations are complex, usually with multi-organ dysfunction, aggressive course, and poor prognosis. The pathogenesis and treatment of HPS should be further studied.