Hepatosplenic alphabeta T-cell lymphomas: a report of 14 cases and comparison with hepatosplenic gammadelta T-cell lymphomas.
Hepatosplenic alphabeta T-cell lymphomas: a report of 14 cases and comparison with hepatosplenic gammadelta T-cell lymphomas.
复制标题
肝脾alphabeta T细胞淋巴瘤14例报告并与肝脾gammadelta T细胞淋巴瘤比较。
DOI：
--
复制
发表时间：
2001
影响因子：
5.6
通讯作者：
J. Cousar
中科院分区：
文献类型：
--
作者：
W. Macon;N. Levy;Paul J. Kurtin;K. E. Salhany;M. Elkhalifa;T. T. Casey;F. Craig;C. Vnencak;M. Gulley;J. P. Park;J. Cousar
肝脾γδT细胞淋巴瘤是一种独特的实体瘤，其特征是发生于年轻成年男性，伴有肝脾肿大、B症状、外周血细胞减少，无淋巴结肿大；脾红髓、肝窦和骨髓窦的淋巴瘤浸润； T 细胞受体 (TCR) γδ 链和细胞毒性 T 细胞表型；等染色体7q；和积极的临床过程。相比之下，本研究描述了 14 例表达 TCR 字母链的肝脾 T 细胞淋巴瘤的临床病理特征。 11 名女性和 3 名男性发生了这种情况，中位年龄为 36 岁。除了女性占多数和年龄分布（5 名患者年龄小于 13 岁，5 名患者年龄大于 50 岁）外，临床表现与之前描述的肝脾 γδ T 细胞淋巴瘤相似。疾病分布主要在脾红髓和肝窦，尽管四例肝脏浸润主要在门静脉周围。在八名患者中观察到骨髓受累通常发生在间质和/或鼻窦内。尽管只有两名患者出现淋巴结肿大，但五名患者的淋巴结受累。 10例由中等大小的肿瘤细胞组成，其细胞核为圆形/椭圆形，染色质稍分散，核仁不明显，细胞质含量不足至中等。四种淋巴瘤主要含有大细胞，具有不规则的细胞核、分散的染色质、可辨别的核仁以及中等至丰富的细胞质。所有 14 例淋巴瘤中的肿瘤细胞均为细胞毒性 Alphata T 细胞； 13 共表达自然杀伤细胞相关抗原并显示 T 细胞克隆性。三种淋巴瘤与 Epstein-Barr 病毒有关。四个病例中有两个具有等染色体 7q。 11 名患者死亡，其中 8 名患者在诊断后一年内死亡，两名患者在联合化疗后仍保持完全缓解。这些数据表明肝脾 T 细胞淋巴瘤包括 Alphata 亚型。该组与先前认识的γδ组一起应被视为同一疾病实体的表型异质亚型。
Hepatosplenic gammadelta T-cell lymphoma is a distinct entity, characterized by occurrence in young adult males with hepatosplenomegaly, B-symptoms, peripheral blood cytopenias, and no lymphadenopathy; lymphomatous infiltrates in the splenic red pulp, hepatic sinusoids, and bone marrow sinuses; T-cell receptor (TCR) gammadelta chains and a cytotoxic T-cell phenotype; isochromosome 7q; and an aggressive clinical course. In comparison, this study describes the clinicopathologic features of 14 hepatosplenic T-cell lymphomas expressing TCR alphabeta chains. They occurred in 11 women and 3 men with a median age of 36 years. Clinical presentation was similar to that described previously for hepatosplenic gammadelta T-cell lymphomas, except for the female preponderance and age distribution (5 patients younger than 13 years of age and 5 patients older than 50 years of age). Disease distribution was primarily in the splenic red pulp and hepatic sinusoids, although liver infiltrates were largely periportal in four cases. Bone marrow involvement, observed in eight patients, was usually interstitial and/or within the sinuses. Lymph nodes were involved in five patients, although lymphadenopathy was demonstrable in only two. Ten cases were composed of intermediate-size tumor cells with round/oval nuclei, slightly dispersed chromatin, inconspicuous nucleoli, and scant to moderate amounts of cytoplasm. Four lymphomas contained primarily large cells with irregular nuclei, dispersed chromatin, discernible nucleoli, and moderate to abundant cytoplasm. Tumor cells in all 14 lymphomas were cytotoxic alphabeta T-cells; 13 co-expressed natural killer cell-associated antigens and showed T-cell clonality. Three lymphomas were associated with Epstein-Barr virus. Two of four cases had an isochromosome 7q. Eleven patients are dead, eight within a year of diagnosis, and two patients have maintained complete remissions after combination chemotherapy. These data show that hepatosplenic T-cell lymphomas include an alphabeta-subtype. This group, along with the previously recognized gammadelta group, should be recognized as phenotypically heterogeneous subtypes of the same disease entity.