摘要目的 分析和总结T淋巴母细胞淋巴瘤/白血病(T-LBL/ALL)的临床、组织学、免疫表型特征,以提高对T-LBL/ALL的认识和诊断水平.方法 采用HE、免疫组织化学(EliVision法)、原位杂交及聚合酶链反应等方法结合临床资料对128例T-LBL/ALL进行了分析.结果 男94例,女34例.男女比2.8:1.年龄4-88岁,平均27岁,中位年龄22岁.58例病变累及淋巴结,27例累及淋巴结外,43例淋巴结内外均有累及.其中73.3%(74/101)累及颈部淋巴结,42.6%(43/101)累及纵隔.病变以弥漫为主,少数呈结节状.多数病例的瘤细胞为中小细胞,少数瘤细胞较大.瘤细胞表达末端脱氧核苷酸转移酶(TdT,94.5%,121/128)、CD34(49.0%,48/98)、CD3(72.2%,78/108)、CD7(96.3%,104/108)、CD43(88.9%,56/63)、CD79a(7.1%,5/70)、CD10(32.9%,25/76)、CD99(96.7%,58/60)、Pax-5(4.4%,4/91);128例髓过氧化物酶(MPO)均为阴性.共随访51例(39.8%),随访时间1～53个月.总体存活率68.6%;总体中位生存时间12个月.不同年龄组中CD3阳性率差异有统计学意义,30岁以上的病例CD3阳性率显著降低.CD10阳性患者的生存时间较阴性患者的生存时间短.5例T细胞受体(TCR)基因重排检测结果显示,4例有TCR基因克隆性重排.结论 T-LBL/ALL主要发生于青少年,以颈部淋巴结肿大和(或)纵隔肿物为主要临床表现.多数病例的肿瘤细胞以中小细胞为主,弥漫分布,但是也应注意到少数病例细胞较大,或结节状生长.免疫组织化学CD7、Pax-5、TdT、CD34、Ki-67五项抗体的联合应用有助于大多数病例确诊.对极少数病例可辅以TCR基因重排检测.

abstractsObjective To clarify clinical and morphological features and immunophenotype of T lymphoblastic lymphoma/leukaemia (T-LBL/ALL)and to further improve the knowledge and diagnostic accuracy for T-ALL/LBL Methods 128 cases of T-LBL/ALL were analyzed for the clinical features,morphology,immunophenotype and TCR gene rearrangement using routine eosin and haematoxylin stain,immunohistochemistry and polymerase chain reaction combining with the clinical findings. Results In 128 cases of T-LBL/ALL,there were 94 male and 34 female. The ratio of male/female was 2. 8: 1. The age of patients ranged from 4 to 88 years,with an average of 27 years and a median of 22 years. Lymph nodes and extranodal areas were involved in 58/128 and 27/128 cases of T-LBL/ALL,respectively. The other 43 cases had involvement of both nodal and extranodal areas. Cervical node and mediastinum were involved in 74 cases and 43 cases,respectively. Diffuse growth pattern of tumor cells was predominant. Nodular growth pattern was seen only in a few cases. Most cases composed of small to medium-sized lymphoblasts,and other 7 cases showed a composition of large lymphoblasts. Tumor cells expressed TdT in 121/128 (94. 5% )cases,CD34 in 48/98(49.0% )cases,CD3 in 78/108(72. 2% )cases,CD7 in 104/108(96. 3% )cases,CD43 in 56/63 (88.9%)cases,CD79a in 5/70(7.1% )cases,CD10 in 25/76 (32.9%)cases,CD99 in 58/60 (96.7% )cases and Pax-5 in 4/91 (4. 4% )cases. All of the cases were negative for MPO. A follow up data,ranging from 1 to 53 months,was obtained in 51/128 (39. 8% )patients. The over all survival rate was 68. 6% and the median survival time was 12 months. Under a similar condition of carrying a positive staining result on CD3 in tumor cells,there was a statistically significant difference between patients in the group of over 30 of age and that with the age ranging from 11 to 30. Patients associating with a CD10 positive staining of tumor cells showed also a shorter survival period. In addition,there were 4 out of 5 cases showing the presence of TCR gene rearrangement. Conclusions T-LBL/ALL are aggressive in behavior,associating mainly with enlarged cervical lymph nodes and masses in the mediastinum,occurring predominantly in children and young adults. Although small to medium-sized tumor cells with diffuse pattern were found in most cases,however,large-sized tumor cells and nodular pattern could also be obtained in a few cases. Immunohistochemistry staining particularly adoption of CD7,Pax-5,TdT,CD34 and Ki-67 stainings in combination are helpful of making a diagnosis for T-LBL/ALL. Aanalysis of TCR gene rearrangement will be helpful for the diagnosis of a few difficult cases.