Guan Chaoyang, Li Jian, Xu Peipei, Ouyang Jian, Chen Bing
Department of Hematology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School Nanjing 210008, P. R. China.
Int J Clin Exp Pathol. 2015 Aug 1;8(8):9629-37. eCollection 2015.
We reported a rare case of composite diffuse large B-cell lymphoma and T lymphoblastic leukemia/lymphoma (T-LBL) in a 46-year-old woman with progressive enlargement of the breast lump. The patient initially sought care at a local hospital with a single left breast lump without any other physical examination findings. Histopathological analysis of which revealed a diffuse infiltration of tumor cells that were rich in cytoplasm with vesicular chromatin and prominent nucleoli. Further analysis of immunohistochemistry showed a cluster of neoplastic cells which express B-cell markers: CD19, CD20 (weak), CD79a, PAX5 and BCL-2, but negative for T-cell markers such as CD2, CD3, CD5 and CD7. PET-CT showed evidence of lymphadenopathy and splenomegaly, which may indicate lymphoma infiltration. Then a biopsy of bone marrow showed typical features of T-LBL. The aberrant terminal deoxynucleotidyl transferase (TDT) and cCD3 positive T-cell population that lack surface CD10 and CD19 were identified by flow cytometric immunophenotyping. Polymerase chain reaction analysis of the T-cell receptor gamma gene and IgH gene revealed a clonal rearrangement and confirming T-cell clonality. Fluorescence in-situ hybridization (FISH) revealed a deletion of the P53 gene in these T-neoplastic cells may indicate a bad outcome of such disease. Neither the large B-cells nor T-cells were positive for Epstein-Barr virus encoded RNA.
我们报告了一例罕见的复合型弥漫性大B细胞淋巴瘤和T淋巴母细胞白血病/淋巴瘤（T-LBL）病例，患者为一名46岁女性，左侧乳房肿块进行性增大。患者最初因左侧乳房单发肿块到当地医院就诊，无其他体格检查异常发现。对该肿块进行组织病理学分析，结果显示肿瘤细胞弥漫浸润，这些细胞胞质丰富，核染色质呈泡状，核仁明显。免疫组化进一步分析显示一群肿瘤细胞表达B细胞标志物：CD19、CD20（弱阳性）、CD79a、PAX5和BCL-2，但T细胞标志物如CD2、CD3、CD5和CD7呈阴性。PET-CT显示有淋巴结病和脾肿大迹象，这可能提示淋巴瘤浸润。随后进行的骨髓活检显示出T-LBL的典型特征。通过流式细胞术免疫表型分析鉴定出异常末端脱氧核苷酸转移酶（TDT）和缺乏表面CD10和CD19的胞质CD3阳性T细胞群。T细胞受体γ基因和IgH基因的聚合酶链反应分析显示存在克隆重排，证实了T细胞的克隆性。荧光原位杂交（FISH）显示这些T肿瘤细胞中P53基因缺失，这可能提示该疾病预后不良。无论是大B细胞还是T细胞，均未检测到爱泼斯坦-巴尔病毒编码RNA阳性。
