CAJ | 학술논문

患者男,51岁,上腹部胀痛4个月,颈项、躯干、双大腿结节、斑块1个月.当地多家医院行胃肠道相关检查,并予对症处理,治疗效果不佳.腹部皮肤结节组织病理检查:真皮下层和皮下脂肪小叶内大量增生的小血管腔内填塞中等偏大异形淋巴样细胞;免疫组化标记:血管腔内异形细胞CD2(+++)、CD4(-)、CD8(-)、CD3ε胞质(++)、CD99(++)、CD43(+++)、CD56(+++)、细胞毒颗粒相关蛋白(TIA-1)(++)、穿孔素(++)、EBER(+++)、CD20(-)、CD79a(-)、CD30(-)、细胞角蛋白(-)、S100(-)、CD68(-)、血管内皮细胞CD31(+)、CD34(+).诊断:以胃肠道症状首发伴有皮肤表现的血管内NK/T细胞淋巴瘤.给予环磷酰胺、长春新碱、柔红霉素、地塞米松+依托泊苷方案化疗,病情得到迅速控制,目前仍在随访中.
환자남,51세,상복부창통4개월,경항、구간、쌍대퇴결절、반괴1개월.당지다가의원행위장도상관검사,병여대증처리,치료효과불가.복부피부결절조직병리검사:진피하층화피하지방소협내대량증생적소혈관강내전새중등편대이형림파양세포;면역조화표기:혈관강내이형세포CD2(+++)、CD4(-)、CD8(-)、CD3ε포질(++)、CD99(++)、CD43(+++)、CD56(+++)、세포독과립상관단백(TIA-1)(++)、천공소(++)、EBER(+++)、CD20(-)、CD79a(-)、CD30(-)、세포각단백(-)、S100(-)、CD68(-)、혈관내피세포CD31(+)、CD34(+).진단:이위장도증상수발반유피부표현적혈관내NK/T세포림파류.급여배린선알、장춘신감、유홍매소、지새미송+의탁박감방안화료,병정득도신속공제,목전잉재수방중.
A 51-year-old man presented with a 4-month history of upper abdominal distending pain and 1-month history of cutaneous nodules and plaques on the neck, trunk and bilateral thighs. The patient underwent many gastrointestinal tract examinations in several local hospitals, and symptomatic treatment did not work. The biopsy of nodules on the abdomen revealed medium- to large-sized atypical lymphoid cells within numerous small vessels in lower dermis and subcutaneous fat tissue. Additionally, the atypical cells were present exclusively within vascular lumina. Immunohistochemical labeling showed the reactivity of neoplastic cells to CD2, CD99, CD3ε, CD43, CD56, Epstein-Barr virus-encoded small nuclear RNAs (EBER), and cytotoxic proteins such as T-cell intracellular antigen-1 (TIA-1) and perforin, but not to CD4, CD8, CD20, CD79a,CD30, cytokeratin (CK), S100, or CD68. The endothelial cells lining the involved vessels exhibited the reactivity to CD31 and CD34. Based on the above findings, the patient was diagnosed with intravascular NK/T-cell lymphoma firstly manifesting as gastrointestinal tract symptom and complicated by skin lesions. Following combined chemotherapy with cyclophosphamide, daunorubicin, vincristine, prednisone and etoposide, the patient experienced a quick and satisfactory recovery and the follow-up still continued.