Yu Bing, Du Jin-rong, Xie Jian-lan, Yu Ran, Zheng Xiao-dan, Zhu Hong, Zhou Xiao-ge
Department of Pathology, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China.
Zhonghua Bing Li Xue Za Zhi. 2010 Jul;39(7):452-7.
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.
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.
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.
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. Analysis of TCR gene rearrangement will be helpful for the diagnosis of a few difficult cases.
阐明T淋巴母细胞淋巴瘤/白血病（T-LBL/ALL）的临床、形态学特征及免疫表型，进一步提高对T-ALL/LBL的认识及诊断准确性。
采用常规伊红和苏木精染色、免疫组织化学及聚合酶链反应，结合临床资料，对128例T-LBL/ALL患者的临床特征、形态学、免疫表型及TCR基因重排进行分析。
128例T-LBL/ALL患者中，男性94例，女性34例，男女比例为2.8∶1。患者年龄4～88岁，平均27岁，中位数22岁。T-LBL/ALL患者中，58例累及淋巴结，27例累及结外区域，43例同时累及淋巴结和结外区域。颈部淋巴结和纵隔受累分别为74例和43例。肿瘤细胞以弥漫性生长方式为主，仅少数病例呈结节状生长方式。多数病例由小至中等大小的淋巴母细胞组成，7例由大淋巴母细胞组成。肿瘤细胞TdT表达阳性121/128例（94.5%），CD34表达阳性48/98例（49.0%），CD3表达阳性78/108例（72.2%），CD7表达阳性104/108例（96.3%），CD43表达阳性56/63例（88.9%），CD79a表达阳性5/70例（7.1%），CD10表达阳性25/76例（32.9%），CD99表达阳性58/60例（96.7%），Pax-5表达阳性4/91例（4.4%）。所有病例MPO均为阴性。128例患者中51例（39.8%）获得1～53个月的随访资料，总生存率为68.6%，中位生存时间为12个月。在肿瘤细胞CD3染色阳性的类似情况下，年龄大于30岁组与11～30岁组患者之间存在统计学差异。肿瘤细胞CD10染色阳性的患者生存期也较短。此外，5例中有4例存在TCR基因重排。
T-LBL/ALL具有侵袭性，主要表现为颈部淋巴结肿大和纵隔肿物，多见于儿童及青年。多数病例为小至中等大小肿瘤细胞弥漫性生长，但少数病例也可见大肿瘤细胞及结节状生长方式。免疫组织化学染色尤其是联合应用CD7、Pax-5、TdT、CD34及Ki-67染色有助于T-LBL/ALL的诊断。TCR基因重排分析有助于少数疑难病例的诊断。
