胃原发间变性大细胞淋巴瘤4例临床病理学特点及预后分析  

Analysis of cUnicopthologic features and prognosis of 4 cases primary gastric anaplastic large cell lymphomas

作　　者：赖玉梅[1] 黄欣[1] 刘翠苓[1] 王小燕[1] 李敏[1] 孙琳[1] 陆伟[1] 高子芬[1] 

机构地区：[1]北京大学医学部基础医学院病理学系血液病理研究室,100191

出　　处：《白血病．淋巴瘤》2012年第4期203-207,共5页Journal of Leukemia & Lymphoma

摘　　要：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、CD3ε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＋白体干细胞移植，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CD3ε阳性率较高，早期诊断和积极治疗有助于改善患者的预后。Objective To evaluate clinicopathologic features and prognosis of primary gastric anaplastic large cell lymphomas （ALCL）. Methods Clinical data and paraffin 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 CD3εwas 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.

关 键 词：淋巴瘤 大细胞 间变性 胃 原发 

分 类 号：R735.2[医药卫生—肿瘤]