儿童鼻型NK/T细胞淋巴瘤1例并文献复习

Child nasal NK/T cell lymphoma:a case report and literature review

目的报道罕见儿童鼻型NK/T细胞淋巴瘤1例。方法总结分析儿童鼻型NK/T细胞淋巴瘤1例的临床及组织病理学特征。详细采集患儿体格检查、鼻窦CT和MRI、骨髓像、免疫学及组织病理学检查等资料,并进行文献复习。结果患儿,男,15岁,因"发热、皮疹及鼻堵1个月"入院。入院查体可见全身散在大小不等皮疹,略高出皮肤,直径1～5cm,呈鲜红色及紫红色,以双下肢多见,左下肢胫骨内侧可见一直径约7cm皮肤结节。鼻翼肿胀,鼻腔内有增生物,有异味及黄色分泌物。鼻窦CT及MRI均提示鼻腔占位性病变。患儿入院后腹痛进行性加重,腹部X线检查提示肠穿孔,行直肠穿孔修补、阑尾切除及回肠提吊造瘘术。左下肢皮疹病理学检查示:真皮小血管及皮肤附件周围可见异形淋巴细胞浸润;左下肢胫骨内侧皮肤结节穿刺涂片见大量肿瘤细胞;切除肠管病理学检查示:部分盲肠壁黏膜及肌间可见灶状异形淋巴细胞浸润,其形态与左下肢皮疹病理学检查中所见相似。免疫组化示:CD45RO(++),CD3(++)～(+++),CD56(++)～(+++);GranzymeB、CD20、CD79a和CD68均(-)。结合临床表现、病理学检查镜下形态及免疫组化结果,确诊为鼻型NK/T细胞淋巴瘤。患儿家属放弃治疗,患儿于出院2个月后死亡。结论鼻型NK/T细胞淋巴瘤主要累及鼻腔及面中线部位,并可表现为多系统受累。患儿如有呈进行性面部中线破坏性病变应高度警惕本病的可能。

Objective By learning the clinical features of child nasal NK/T cell lymphoma to enhance the ability of early diagnosis and treatment.Methods One case of child with nasal NK/T cell lymphoma wAS reported in the clinical features,histopathological features based on disease history,physical examination,sinus CT and MRI,bone marrow cytology,immunology and histopathological examinations of a new nasal tissue,skin and subcutaneous tissue,cecum mucosa and muscle.Then the case was followed up and the literatures were reviewed.Results The case was a 15 years old boy.He came to hospital because of fever,rash and blocked nose for 1 month.The child sufferred from nasal cavity tumors with skin involvement,as well as digestive system and joint metastasis.CT and MRI tests of the nasal cavity showed a space-occupying lesion.The pathology of left lower extremity skin nodules,bowel and left anterior tubercle puncture indicated that profiled lymphocyte infiltration could be seen surrounding small blood vessels of dermis and skin appendage.Immunohistochemistry showed tumor cells expressed T cell marker CD45RO,CD3 and NK-cell-specific antibody CD56.Cytotoxic molecule was positive,such as perforin,TIA-1,Granzyme B and so on.It was proved to be nasal NK/T cell lymphoma by clinical manifestations,microscopic morphology and immunohistochemical characteristics.As his parents gave up the treatment after the diagnosis,the child died in 2 months after discharge.Conclusions Child nasal NK/T cell lymphoma was a rare and special type of primary extranodal non-Hodgkin＇s lymphoma.The disease mainly affected the nasal cavity and the facial midline or presented as multi-system involvement.The disease is highly aggressive with rapid progress.It was easily resistant to chemotherapeutic drug and had poor prognosis in advanced cases.Early clinical manifestations were atypical and it was often misdiagnosed as inflammation,Wegner granulomatosis,other types of lymphoma and Behcet＇s disease.