CAJ | 학술논문

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时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(morphologie M)、免疫学(immunophenotypie,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.
자연살상세포(Natural killer,NK)/T세포림파류시일류한견림파류.기원우활화적NK세포혹활화적T세포.타통상위결외병변,왕왕표현위위우면부중선조직적파배성병조.NK/T세포림파류상견우비강급비두,차외기타결외기관여인부、위장도、고환등부위적침범야유보도,야유일사한견적표현위흉강적액적보도.본문중보도료1명이흉강적액위수발증상적NK/T세포림파류.저명환자재외원통과시흉막활검조직병이학급면역조화염색오진위B세포형비곽기금림파류.입원후통과대흉수세포형태학(morphologie M)、면역학(immunophenotypie,I)、세포유전학(cytogenetic C)급분자생물학(molecular,M)(MICM)연합검측.결과표명,형태학검측증실유유치림파세포,류식세포술검측발현저사세포표체포장CD3(cCD3)급CD56,분자생물학검측유극륭성T세포수체γ(TCR-γ)기인중배,종이규정오진이정학장기진단위NK/T세포림파류,병성공지진행료이기인조혈간세포이식.결론:대저한견병례응강조MICM연합진단기술대진단적중요성,령외,차한견질병유특수림상표현,능구대기진행성공적치료.
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, immu nophenotypic, 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.