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9例γδT细胞淋巴瘤或白血病的临床及实验室特征

[The clinical and laboratory features of 9 cases with gammadeltaT cell lymphoma or leukemia].

作者信息

Tong Chun-Rong, Wang Hui, Lin Yue-Hui, Yang Jun-Fang, Zhao Jie, Zhang Xian, Yin Yu-Ming, Fei Xin-Hong, Wu Ping, Wang Tong, Liu Hong-Xing, Cai Peng, Wei Zhi-Jie, Xiao Juan, Liu De-Yan, Xiong Min, Zhong Jing, Lu Dao-Pei

机构信息

Beijing Daopei Hospital, Beijing 100049, China.

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2011 Jul;32(7):445-9.

Abstract

OBJECTIVE

To analyze the clinical and laboratory features of 9 cases of gammadeltaT cell lymphoma or leukemia.

METHODS

From 2007 to 2011, 9 patients with gammadeltaT-cell lymphoma/leukemia were diagnosed in our hospital. The immunophenotype of the abnormal cells were detected by flow cytometry, clonal gene rearrangement of IgH, TCRgamma, TCRdelta by PCR, chromosome karyotype analysis by G banding, acute leukemia gene and the DNA of type 1 - 8 human herpes virus by multiple nested PCR, The gammadeltaT cells were determined by T cell with TCR gammadelta chain, the malignant gammadelta T cells by the abnormal expression of T cell antigens and the precursor malignant gammadelta T cells by the expression of CD34, TDT, CD99, CD1 a or acute leukemia genes.

RESULTS

In the 9 patients with gammadeltaT cell lymphoma leukemia, significant malignant gammadeltaT cells infiltration of bone marrow were found in 8 with blast morphology. 5 were diagnosed as T-ALL/LBL (gammadeltaT type) and 4 HSgammadelta TCL. The clonal gene rearrangement of TCRgamma and/or TCRB were detected in 6/6 patients. Patients either did not achieve complete remission(CR) after induction therapy or relapsed quickly after CR. Only 4/5 patients remained continuous CR(CCR) at 2, 2, 3,12 months respectively, after allogeneic hematopoietic stem cell transplantation (allo-HSCT), the fifth T-ALL (gammadeltaT) relapsed 1 month after allo-HSCT.

CONCLUSIONS

The incidence of gammadelta T cell lymphoma or leukemia may be higher than reported, part of them were T-ALL/LBL with poor prognoses. FCM and clonal gene rearrangement of TCRgamma and/or TCRdelta are helpful to diagnosis. Allo-HSCT may be the only curative approach.

摘要

目的

分析9例γδT细胞淋巴瘤或白血病的临床及实验室特征。

方法

2007年至2011年,我院诊断9例γδT细胞淋巴瘤/白血病患者。采用流式细胞术检测异常细胞免疫表型,聚合酶链反应(PCR)检测免疫球蛋白重链(IgH)、T细胞受体γ链(TCRγ)、T细胞受体δ链(TCRδ)克隆基因重排,G显带分析染色体核型,多重巢式PCR检测急性白血病相关基因及1 - 8型人类疱疹病毒DNA。通过带有TCRγδ链的T细胞确定γδT细胞,通过T细胞抗原异常表达确定恶性γδT细胞,通过CD34、末端脱氧核苷酸转移酶(TDT)、CD99、CD1a表达或急性白血病相关基因确定前体恶性γδT细胞。

结果

9例γδT细胞淋巴瘤白血病患者中,8例骨髓有明显恶性γδT细胞浸润,呈原始细胞形态。5例诊断为T淋巴母细胞白血病/淋巴瘤(γδT型),4例为皮下脂膜炎样γδT细胞淋巴瘤。6/6例患者检测到TCRγ和/或TCRB克隆基因重排。患者诱导治疗后未达完全缓解(CR)或CR后很快复发。异基因造血干细胞移植(allo - HSCT)后,仅4/5例患者分别在2、2、3、12个月时保持持续完全缓解(CCR),第5例T淋巴母细胞白血病(γδT型)allo - HSCT后1个月复发。

结论

γδT细胞淋巴瘤或白血病发病率可能高于报道,部分为预后不良的T淋巴母细胞白血病/淋巴瘤。流式细胞术及TCRγ和/或TCRδ克隆基因重排有助于诊断。allo - HSCT可能是唯一的治愈方法。

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