CAJ | 학술논문

目的探讨胃原发间变性大细胞淋巴瘤( ALCL)的临床病理学特点及预后.方法收集经北京大学医学部基础医学院病理学系确诊的4例胃原发ALCL,通过HE染色、免疫组织化学、EB病毒( EBV)编码的小核RNA(EBV-EBER)原位杂交、间期荧光原位杂交技术并结合文献复习,分析其临床病理学特点及预后.结果男性3例,女性1例；年龄27～87岁,中位年龄58.5岁.大体标本均为胃部巨大溃疡型肿物；镜下见肿瘤细胞多形性明显、体积大的肿瘤细胞弥漫性浸润并破坏胃壁.肿瘤细胞一致性强表达LCA和CD30、CD3e阳性率75％(3/4).ALK蛋白水平及基因水平检测均为阴性(4/4),亦未检测到EBV感染.2例患者接受手术+CHOP方案化疗,1例接受CHOP方案化疗+自体干细胞移植,此3例患者均获得临床完全缓解出院,随访截至2011年11月30日,均未出现肿瘤复发或进展.另1例患者未接受治疗,于确诊后22个月死亡.结论胃原发ALCL多为ALK阴性,其临床病理学特点及预后与其他部位原发的ALK阴性ALCL基本相同,但CD3e阳性率较高,早期诊断和积极治疗有助于改善患者的预后.
목적 탐토위원발간변성대세포림파류( ALCL)적림상병이학특점급예후.방법 수집경북경대학의학부기출의학원병이학계학진적4례위원발ALCL,통과HE염색、면역조직화학、EB병독( EBV)편마적소핵RNA(EBV-EBER)원위잡교、간기형광원위잡교기술병결합문헌복습,분석기림상병이학특점급예후.결과 남성3례,녀성1례；년령27～87세,중위년령58.5세.대체표본균위위부거대궤양형종물；경하견종류세포다형성명현、체적대적종류세포미만성침윤병파배위벽.종류세포일치성강표체LCA화CD30、CD3e양성솔75％(3/4).ALK단백수평급기인수평검측균위음성(4/4),역미검측도EBV감염.2례환자접수수술+CHOP방안화료,1례접수CHOP방안화료+자체간세포이식,차3례환자균획득림상완전완해출원,수방절지2011년11월30일,균미출현종류복발혹진전.령1례환자미접수치료,우학진후22개월사망.결론 위원발ALCL다위ALK음성,기림상병이학특점급예후여기타부위원발적ALK음성ALCL기본상동,단CD3e양성솔교고,조기진단화적겁치료유조우개선환자적예후.
Objective To evaluate clinicopathologic features and prognosis of primary gastric anaplastic large cell lymphomas (ALCL).Methods Clinical data and parafiin blocks of 4 patients diagnosed with primary gastric ALCL were obtained. The diagnosis of all cases was based on the criteria of WHO classification of hematolymphoid neoplasm.Furthermore,chromosomal rearrangement involving ALK gene was detected by interphase fluorescence in situ hybridization (FISH) and Epstein-Barr virus (EBV) status was determined by in situ hybridization(ISH) for EBV-encoded small RNAs (EBERs).Results The patients (3 males and 1 female) were from 27 to 87 years old, with a median age of 58.5 years. All the four cases presented with a solitary ulcerative mass in stomach. Morphologically, the normal architecture of gastric wall was effaced by the diffuse infiltration of tumor cells in which the characteristic hallmark cells were easily identified.The tumor cells of all cases showed a consistently strong expression of LCA and CD30,and CD3e was expressed in 3 of the 4 cases.Both ALK expression and ALK gene rearrangements were negative in all cases.Two cases underwent total or partial gastrectomy followed by CHOP chemotherapy. Another one patient was treated with chemotherapy and autologous stem cell transplantation. None of these 3 patients developed a relapse or progression till the last follow-up on Nov 30,2011. While the rest one patient refused to take any treatment and died 20 months after diagnosis. Conclusions Primary gastric ALCL is very rare and usually ALK negative. Its pathologic features as well as the clinical outcome are quite similar to the ALK negative ALCL from other sites, except the more frequently positive CD3e Early diagnosis and proper therapy are of great significance to the prognosis.