CAJ | 학술논문

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1～5 cm,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7 cm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.
목적 보도한견인동비형NK/T세포림파류1례.방법 총결분석인동비형NK/T세포림파류1례적림상급조직병이학특정.상세채집환인체격검사、비두CT화MRI、골수상、면역학급조직병이학검사등자료,병진행문헌복습.결과 환인,남,15세,인"발열、피진급비도1개월"입원. 입원사체가견전신산재대소불등피진,략고출피부,직경1～5 cm,정선홍색급자홍색,이쌍하지다견,좌하지경골내측가견일직경약7 cm피부결절.비익종창,비강내유증생물,유이미급황색분비물.비두CT급MRI균제시비강점위성병변.환인입원후복통진행성가중,복부X선검사제시장천공,행직장천공수보、란미절제급회장제조조루술.좌하지피진병이학검사시:진피소혈관급피부부건주위가견이형림파세포침윤;좌하지경골내측피부결절천자도편견대량종류세포;절제장관병이학검사시:부분맹장벽점막급기간가견조상이형림파세포침윤,기형태여좌하지피진병이학검사중소견상사.면역조화시:CD45RO(++),CD3(++)～(+++), CD56(++)～(+++);GranzymeB、CD20、CD79a화CD68균(-).결합림상표현、병이학검사경하형태급면역조화결과 ,학진위비형NK/T세포림파류.환인가속방기치료,환인우출원2개월후사망.결론 비형NK/T세포림파류주요루급비강급면중선부위,병가표현위다계통수루.환인여유정진행성면부중선파배성병변응고도경척본병적가능.
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.