以胸腔积液为首发症状的NK/T细胞淋巴瘤:MICM技术对诊断的重要性(英文)  

Natural Killer/T Cell Lymphoma Initiating with Pleural Effusion:the Significance of MICM Combined Techniques for the Diagnosis

作　　者：刘芳[1] 林跃辉[1] 刘红星[1] 王彤[1] 单福香[1] 童春荣[1] 陆道培[1] 

机构地区：[1]北京市道培医院移植科,北京100049

出　　处：《中国实验血液学杂志》2009年第5期1347-1351,共5页Journal of Experimental Hematology

摘　　要：自然杀伤细胞(Natural killer,NK)/T细胞淋巴瘤是一类罕见淋巴瘤,起源于活化的NK细胞或活化的T细胞。它通常为结外病变,往往表现为位于面部中线组织的破坏性病灶。NK/T细胞淋巴瘤常见于鼻腔及鼻窦,此外其他结外器官如咽部、胃肠道、睾丸等部位的侵犯也有报导,也有一些罕见的表现为胸腔积液的报导。本文中报道了1名以胸腔积液为首发症状的NK/T细胞淋巴瘤。这名患者在外院通过对胸膜活检组织病理学及免疫组化染色误诊为B细胞型非霍奇金淋巴瘤。入院后通过对胸水细胞形态学(morphologic M)、免疫学(immunophenotypic,I)、细胞遗传学(cytogenetic C)及分子生物学(molecular,M)(MICM)联合检测。结果表明,形态学检测证实有幼稚淋巴细胞,流式细胞术检测发现这些细胞表达胞浆CD3(cCD3)及CD56,分子生物学检测有克隆性T细胞受体γ(TCR-γ)基因重排,从而纠正误诊而正确将其诊断为NK/T细胞淋巴瘤,并成功地进行了异基因造血干细胞移植。结论:对这罕见病例应强调MICM联合诊断技术对诊断的重要性,另外,此罕见疾病有特殊临床表现,能够对其进行成功的治疗。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 cytoplastic CD3 （ cCD3 ） and CD56 by flow cytometry analysis and had a clonal T cell receptor gamma （TCR-γ） 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.

关 键 词：NK/T细胞淋巴瘤 MICM技术 流式细胞术 T细胞受体Γ基因重排 异基因造血干细胞移植 

分 类 号：R733.1[医药卫生—肿瘤] R730.4[医药卫生—临床医学]