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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 lymphoma is a rare type of lymphoma that originates from activated NK cells or activated T cells. It is usually an extranodal disease, often manifested as a devastating lesion in the midline tissue of the face. NK / T-cell lymphoma is common in the nasal and sinus, in addition to other extranodal organs such as the throat, gastrointestinal tract, testicular and other parts of the invasion has also been reported, there are some rare manifestations of pleural effusion. This article reported a pleural effusion as the first symptom of NK / T cell lymphoma. The patient was misdiagnosed as a B-cell non-Hodgkin’s lymphoma by pleural biopsy histopathology and immunohistochemistry in the hospital. After admission, we detected the combination of morphologic M, immunophenotypic (I), cytogenetic C and molecular (M) (MICM). The results showed that lymphocytes were confirmed by morphological examination. Cytosolic CD3 (cCD3) and CD56 were detected by flow cytometry. Clonal T cell receptor gamma (TCR-γ) gene rearrangements were detected by molecular biology , So as to correct misdiagnosis and correct diagnosis of NK / T cell lymphoma, and successfully carried out allogeneic hematopoietic stem cell transplantation. CONCLUSIONS: The importance of the combination of the MICM diagnostic technique for diagnosis in this rare case should be emphasized. In addition, this rare disease has special clinical manifestations that allow it to be successfully treated.