摘要
目的报告9例γδ细胞淋巴瘤／白血病患者的临床及实验室特征。方法对2007至2010年住院诊治的患者，常规询问病史，进行体检及实验室检测。对有异常细胞的骨髓或脾组织用流式细胞分析技术（FCM）行免疫分型，同时用PCR法检测TCRγ／、TCRδ基因克隆性重排，用G显带技术分析染色体，用多重筑巢式PCR技术筛查急性白血病基因及1～8型人类疱疹病毒基因，并行形态学及细胞化学染色。根据表达TCR78链来确定γδBT细胞，根据T细胞抗原表达异常确定恶性γδT细胞，根据γδBT细胞表达CD34、CD99、TDT、CD1a及急性白血病基因等确定为前体γδT细胞。结果共9例患者经FCM分析诊断为γδT细胞淋巴瘤／白血病，初诊时8例患者骨髓中检测出大量恶性细胞，细胞形态同原始细胞。5例患者诊断为急性T淋巴细胞白血病（T—ALL）或淋巴母细胞淋巴瘤（LBL）（γδT型），4例患者诊断为肝脾γδT细胞淋巴瘤（HSγδTCL）。行TCR基因克隆重排检测的6例患者均检测出有克隆性TCRγ／和（或）TCRB基因重排。全部患者化疗难以完全缓解（CR）或CR后很快复发，5例接受异基因造血干细胞移植（allo—HSCT）的患者4例持续CR，CR时间分别为2、2、3、12个月；1例T—ALL（γδST型）患者在allo—HSCT后1个月内复发。结论yST细胞淋巴瘤／白血病的发病率可能比既往报道的高，部分T—ALL／LBL也为γδT细胞型；FCM是快速可靠的诊断方法，克隆性TCRγ和（或）TCRδ基因重排阳性有助于诊断；患者预后差，allo—HSCT可能是唯一可治愈的方法。
Objective To analyze the clinical and laboratory features of 9 cases of γδT cell lymphoma or leukemia. Methods From 2007 to 2011,9 patients with γδT-cell lymphoma/leukemia were diagnosed in our hospital. The immunophenotype of the abnormal cells were detected by flow cytometry, clonal gene rearrangement of IgH, TCRγ,TCRδ by PCR, chromosome karyotype analysis by G banding, acute leukemia gene and the DNA of type 1 - 8 human herpes virus by mulplex nested PCR, The γδT cells were determined by T cell with TCR γδ chain, the malignant γδT ceils by the abnormal expression of T cell antigens and the precursor malignani γδT cells by the expression of CD34, TDT , CD99, CD1 a or acute leukemia genes. Results In the 9 patients with γδT cell lymphoma leukemia, significant malignant γδT cells infiltration of bone marrow were found in 8 with blast morphology. 5 were diagnosed as T-ALL/LBL （γδT type ） and 4 HSγδ TCL. The clonal gene rearrangement of TCRγ and/or TCRδ were detected in 6/6 patients. Patients either did not achieve complete renfission（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（γδT） relapsed 1 month after allo-HSCT . Conclusions The incidence of γδ 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 TCRγ and/or TCRδ are helpful to diagnosis. Allo-HSCT may be the only curative approach.
出处
《中华血液学杂志》
CAS
CSCD
北大核心
2011年第7期445-449,共5页
Chinese Journal of Hematology
