9例γδT细胞淋巴瘤/白血病患者的临床及实验室特征
9례γδT세포림파류/백혈병환자적림상급실험실특정
The clinical and laboratory features of 9 cases with 76T cell lymphoma or leukemia
  • 万方数据
    中华血液学杂志 중화혈액학잡지
    Chinese Journal of Hematology
    2011年 7期 445-449 ,共5页

    童春容%王卉%林跃辉%杨君芳%赵杰%张弦%殷宇明%费新红%伍平%王彤%刘红星%蔡鹏%魏志杰%肖娟%刘德琰%熊敏%钟静%陆道培

    동춘용%왕훼%림약휘%양군방%조걸%장현%은우명%비신홍%오평%왕동%류홍성%채붕%위지걸%초연%류덕염%웅민%종정%륙도배

    淋巴瘤,γδT细胞%白血病,T细胞,急性%流式细胞术%基因重排 淋巴瘤,γδT細胞%白血病,T細胞,急性%流式細胞術%基因重排
    림파류,γδT세포%백혈병,T세포,급성%류식세포술%기인중배
    Lymphoma,γδT cell%Leukemia,T-cell,acute%Flow cytometry%Gene rearrangement
    目的 报告9例γδ T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TCRγδ链来确定γδT细胞,根据T细胞抗原表达异常确定恶性γδT细胞,根据γδT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)TCRδ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TALL(γδT型)患者在allo-HSCT后1个月内复发.结论 γδT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.
    목적 보고9례γδ T세포림파류/백혈병환자적림상급실험실특정.방법 대2007지2010년주원진치적환자,상규순문병사,진행체검급실험실검측.대유이상세포적골수혹비조직용류식세포분석기술(FCM)행면역분형,동시용PCR법검측TCRγ、TCRδ기인극륭성중배,용G현대기술분석염색체,용다중축소식PCR기술사사급성백혈병기인급1-8형인류포진병독기인,병행형태학급세포화학염색.근거표체TCRγδ련래학정γδT세포,근거T세포항원표체이상학정악성γδT세포,근거γδT세포표체CD34、CD99、TDT、CD1a급급성백혈병기인등학정위전체γδT세포.결과 공9례환자경FCM분석진단위γδT세포림파류/백혈병,초진시8례환자골수중검측출대량악성세포,세포형태동원시세포.5례환자진단위급성T림파세포백혈병(T-ALL)혹림파모세포림파류(LBL)(γδT형),4례환자진단위간비γδT세포림파류(HSγδTCL).행TCR기인극륭중배검측적6례환자균검측출유극륭성TCRγ화(혹)TCRδ기인중배.전부환자화료난이완전완해(CR)혹CR후흔쾌복발,5례접수이기인조혈간세포이식(allo-HSCT)적환자4례지속CR,CR시간분별위2、2、3、12개월;1례TALL(γδT형)환자재allo-HSCT후1개월내복발.결론 γδT세포림파류/백혈병적발병솔가능비기왕보도적고,부분T-ALL/LBL야위γδT세포형;FCM시쾌속가고적진단방법,극륭성TCRγ화(혹)TCRδ기인중배양성유조우진단;환자예후차,allo-HSCT가능시유일가치유적방법.
    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 γδ T cells by the abnormal expression of T cell antigens and the precursor malignant γδ T cells by the expression of CD34, TDT ,CD99, CD1a or acute leukemia genes.Results In the 9 patients with γδT cell lymphoma leukemia, significant malignantγδT 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 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(γδT) relapsed 1 month after allo-HSCT .Conclusion s 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.
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