眼眶原发性NK-T细胞淋巴瘤1例并文献复习.docVIP

眼眶原发性NK/T细胞淋巴瘤1例并文献复习【摘要】目的提高对结外鼻型NK/T细胞淋巴瘤的认识。方法报告1例原发于眼眶的NK/T细胞淋巴瘤，结合文献进行分析讨论。结果患者曾被误诊为眼眶炎性假瘤，后经免疫组化确诊为眼眶NK/T细胞淋巴瘤。肿瘤细胞免疫表型为CD56+,CD2+, CD45RO+, CD79a+, CD20,CD21, CD38, CD138。应用放疗及化疗，患者未能完全缓解，病程中合并噬血细胞综合征，最后死亡。结论眼眶原发性NK/T细胞淋巴瘤罕见，容易误诊，侵袭性强，确诊手段为免疫组化，治疗效果差，预后不良。 【关键词】眼眶肿瘤; NK/T细胞;淋巴瘤 Primary orbital natural killer/T cell lymphoma: a case report and review of the literatureZHAO Jianbao, CHEN ChenDepartment of Hematology，Zaozhuang municipal hospital,Zaozhuang 277100,China 【Abstract】ObjectiveTo well recognize the clinical features of extranodal NK/T cell lymphoma(NKTCL) of nasal type MethodsOne case of primary orbital NK/T cell lymphoma was reported, and the literatures were reviewed ResultsPatient was ever misdiagnosed as inflammatory pseudotumor of orbit, and diagnosed finally by immunohistochemical staining The immunophenotype result showed: CD2+, CD56+,CD45RO+,CD20,CD21 Patient failed to achieve a complete response with both chemotherapy and radiotherapy, and died of lymphoma with hemophagocytic syndrome ConclusionOrbital NK/T cell lymphoma is rare disease easily misdiagnosed with aggressive behavior, poor prognosis and lack of efficient therapies The final diagnosis of this disease depends on immunohistochemical staining. 【Key words】Orbital neoplasma; NK/T cell; Lymphoma 结外鼻型NK/T细胞淋巴瘤(extranodal NK/T cell lymphoma, nasal type)为少见的恶性肿瘤, 原发于眼眶者更为罕见，侵袭性强，破坏性大，极易误诊，目前尚缺乏理想的治疗措施，预后较差［1］。现报告1例眼眶原发性NK/T细胞淋巴瘤病例，并复习相关文献，以提高对结外鼻型NK/T细胞淋巴瘤临床病理特征及诊治方法的认识。 1病例资料 患者，女，54岁，因反复左眼上睑红肿5月入院。患者5 作者单位：277100枣庄市立医院血液科 月前因左眼上睑红肿，发热，体温38℃～39℃，无明显疼痛及眼部分泌物增多，以血管炎待排在本院风湿免疫科住院治疗，血常规、血沉，血生化、自身免疫相关抗体，胸部X线、腹部B超等检查均未见明显异常，核磁共振检查提示左眼眶部占位，应用小剂量甲强龙治疗，眼部症状有所减轻，体温仍38℃左右，无咳嗽及憋喘。到山东省某医院取病理考虑炎性假瘤，予甲强龙500 mg冲击3 d，效果理想，眼睑肿胀消退，体温恢复正常，停药2 d后眼睑红肿复发，再次体温升高。在北京同仁医院手术切除眼眶肿块，病理诊断为：左眼眶内NK/T细胞淋巴 瘤，免疫组化示：CD2+, CD45RO+, CD20, CD79a+, CD38, CD138, CD56+, CD21, Ki67增殖指数80%； TB；EBV+；确诊为原发性左眼眶内NK/T细胞淋巴瘤。于20114月来本院血液科住院治疗。入院查体见：T 378℃, P 81次/min,R 19次/min,BP110/70 mm Hg，中年女性，神志清，