Liu Fang, Lin Yue-Hui, Liu Hong-Xing, Wang Tong, Shan Fu-Xiang, Tong Chun-Rong, Lu Dao-Pei
Department of Transplantation, Beijing Dao-Pei Hospital, Beijing 100049, China.
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2009 Oct;17(5):1347-51.
Natural killer (NK)/T-cell lymphomas represent a rare type of lymphoma derived from either activated NK cells or cytotoxic T cells. They are most commonly extranodal and tend to present as destructive lesions within the midline facial structures. Other than the nasal cavity and Para nasal sinuses, several other extra nodal sites of involvement have been reported, including the pharynx, gastrointestinal tract, and testis. Occasionally, pleural effusion has also been observed. Here, a case of lymphoma of NK/T-cell type presented as pleural effusion was reported. The patient was previously misdiagnosed as B cell non-Hodgkin's lymphoma by pathological and immunohistochemistry (IH) analysis for pleural membrane biopsy specimen. After the analysis of the pleural fluid cells by a combination of morphologic, immunophenotypic, cytogenetic and molecular (MICM) methods in Beijing Dao-Pei hospital, some lymphoblasts were found morphologically, which expressed cytoplasmic CD3 (cCD3) and CD56 by flow cytometry analysis and had a clonal T-cell receptor gamma (TCR-gamma) gene rearrangement by molecular analysis, so that the diagnosis was finally corrected as NK/T-cell lymphoma and an allogeneic stem cell transplantation was successfully performed. In conclusion, this unusual case highlights the significance of MICM combined techniques for the diagnosis of lymphoma, as well as an unusual presentation of a rare disease and the successful treatment.
自然杀伤(NK)/T细胞淋巴瘤是一种罕见的淋巴瘤,起源于活化的NK细胞或细胞毒性T细胞。它们最常见于结外,往往表现为中线面部结构内的破坏性病变。除鼻腔和鼻窦外,还报道了其他几个结外受累部位,包括咽部、胃肠道和睾丸。偶尔也会观察到胸腔积液。在此,报告一例以胸腔积液为表现的NK/T细胞型淋巴瘤病例。该患者先前因胸膜活检标本的病理和免疫组织化学(IH)分析被误诊为B细胞非霍奇金淋巴瘤。在北京道培医院通过形态学、免疫表型、细胞遗传学和分子(MICM)方法相结合对胸水细胞进行分析后,形态学上发现一些淋巴母细胞,流式细胞术分析显示其表达胞质CD3(cCD3)和CD56,分子分析显示有克隆性T细胞受体γ(TCR-γ)基因重排,最终诊断得以纠正为NK/T细胞淋巴瘤,并成功进行了异基因干细胞移植。总之,这个不寻常的病例突出了MICM联合技术在淋巴瘤诊断中的重要性,以及一种罕见疾病的不寻常表现和成功治疗。