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伴噬血细胞综合征的T细胞淋巴瘤临床特点及生存分析 被引量:6

Clinical features and prognostic analysis of T-cell lymphoma with hemophagocytic syndrome
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摘要 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(x。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20d。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(Y一35.05,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。 Objective To investigate the clinical characteristics and survival analysis of T-cell lymphoma with hemophagoeytie 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 (X2 = 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 (Xa = 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.
出处 《白血病.淋巴瘤》 CAS 2013年第5期272-274,277,共4页 Journal of Leukemia & Lymphoma
关键词 淋巴细胞组织增多症 噬血细胞性 淋巴瘤 T细胞 临床特点 治疗 预后 Lymphohistioeytisis, hemophagocytie Lympboma, T-cell Clinical features Treatment Prognosis
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  • 1李彩霞,王艳,姚小敏.疟原虫引起感染相关性噬血细胞综合征一例并文献复习[J].江西医学检验,2006,24(5):477-477. 被引量:1
  • 2James WV, William JG. Hemophagocytic lvmphohistioeytosis diagnosis, pathophysiology, treatment, and future perspectives. Ann Med, 2006, 38 : 20-31.
  • 3Hcnter JI, Home A, Arico M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer, 2007, 48:124-131.
  • 4Janka GE. Familial and acquired hemophagocytie lymphohistiocylosis. Eur J Pedialr, 2007, 166: 95-109.
  • 5Teng CL, Hwang GY, Lee B J, et al. Pregnancy-induced hemophagocytic lymphohistiocytosis combined with autoimmune hemolytic anemia. J Chin Med Assoc, 2009, 72: 156-159.
  • 6Allen CE, Yu X, Kozinetz CA, et al. Highly elevated ferritin levels and the diagnosis of hemophagocytie lymphohistiocytosis. Pediatr Blood Cancer, 2008, 50: 1227-1235.
  • 7Chuang HC, Lay JD, Chuang SE, et al. Epstein-Barr virus (EBV) latent membrane protein-1 down-regulates tumor necrosis factor-alpha (TNF-alpha) receptor-1 and confers resistance to TNF-alpha-induced apoptosis in T cells: implication for the progression to T-cell lymphoma in EBV-associated hemophagocytic syndrome. Am J Pathol, 2007, 170: 1607-1617.
  • 8Henter JI, Home AC, Arico M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pedisr Blood Cancer, 2007,48:124-131.
  • 9Zur SU, Beutel K, Kolbcrq S, et al. Mutation spectrum in children with primary hemophagocytic lymphohistiocytosis: molecular and functional analyses of PRF1,UNC13D STXll, and RAB27A. Hum Mutat, 2006, 27: 62-68.
  • 10Ma KA, Frayer RW, Filipovich AH, et al. Aberrant maturation of mutant perforin underlies the clinical diversity of hemophagocytic lymphohistiocytesis. Clin Invest, 2006, 116:182-192.

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  • 1陈捷,吴颖,田红.淋巴瘤相关性嗜血细胞综合征28例临床诊治分析[J].实用医学杂志,2007,23(17):2714-2716. 被引量:8
  • 2佟红艳,肖峰,张凤娟,刘辉,戴铁颖,孟海涛,钱文斌,麦文渊,金洁.伴噬血细胞综合征的外周T细胞淋巴瘤临床特点及生存分析[J].中华血液学杂志,2007,28(10):698-699. 被引量:6
  • 3Janka GE.Familial and acquired hemophagocytic lymphohistiocytosis[J].Annu Rev Med,2012,63:233-246.
  • 4Bode SF,Lehmberg K,Maul-Pavicic A,etal.Recent advances in the diagnosis and treatment of hemophagocytic lymphohistiocytosis[J].Arthritis Res Ther,2012,14(3):213.
  • 5Henter JI,Horne A,Arico M,etal.HLH-2004:Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis[J].Pediatr Blood Cancer,2007,48(2):124-131.
  • 6Han AR,Lee HR,Park BB,etal.Lymphoma-associated hemophagocytic syndrome:clinical features and treatment outcome[J].Ann Hematol,2007,86(7):493-498.
  • 7Tong H,Ren Y,Qian W,et al.Clinicopathological study on peripheral T-cell non-Hodgkin lymphoma with bone marrow involvement:a retrospective analysis from China[J].IntJ Hematol,2009,90(3):303-310.
  • 8Tong H,Ren Y,Liu H,et al.Clinical characteristics of T-cell lymphoma associated with hemophagocytic syndrome:comparison of T-cell lymphoma with and without hemophagocytic syndrome[J].Leuk Lymphoma,2008,49(1):81-87.
  • 9Yu JT,Wang CY,Yang Y,etal.Lymphoma-associated hemophagocytic lymphohistiocytosis:experience in adults from a single institution[J].Ann Hematol,2013,92(11):1529-1536.
  • 10Takahashi N,Chubachi A,Kume M,etal.A clinical analysis of 52 adult patients with hemophagocytic syndrome:the prognostic significance of the underlying diseases[J].IntJ Hematol,2001,74(2):209-213.

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