CAJ | 학술논문

目的：探讨具有弥漫性大B细胞淋巴瘤（DLBCL）和经典型霍奇金淋巴瘤（CHL）中间特点的灰区淋巴瘤的临床和病理特征，旨在深化对该类交界性B细胞肿瘤的认识。方法回顾性分析16例典型病例的临床资料、组织学形态和免疫组织化学表型。结果16例患者男女比为1.7∶1.0，平均年龄40.2岁。8例表现为外周淋巴结病灶，5例表现为纵隔受累。根据组织学形态和免疫表型特点将该组病变分为三种模式。模式1：4例，形态学上类似于CHL，但肿瘤细胞CD20弥漫强阳性；模式2：8例，形态学上类似于DLBCL，但肿瘤细胞异质性表达CD20、PAX5，同时表达CD30和/或CD15；模式3：4例，形态特点介于CHL和DLBCL之间，肿瘤细胞不同程度地表达CD20、CD30和CD15。11例受检病例中，6例肿瘤细胞表达EB病毒潜伏膜蛋白1。临床上，绝大多数患者对联合利妥昔单抗和CHOP（ R-CHOP）方案的免疫化疗不敏感。结论通过描述三种常见的组织学模式，提出了具有DLBCL和CHL中间特点的灰区淋巴瘤的诊断标准。这类肿瘤外周型病变和纵隔病变的病理特点似有不同。目前对这组交界性肿瘤尚无有效治疗方法，患者预后较差。
목적：탐토구유미만성대B세포림파류（DLBCL）화경전형곽기금림파류（CHL）중간특점적회구림파류적림상화병리특정，지재심화대해류교계성B세포종류적인식。방법회고성분석16례전형병례적림상자료、조직학형태화면역조직화학표형。결과16례환자남녀비위1.7∶1.0，평균년령40.2세。8례표현위외주림파결병조，5례표현위종격수루。근거조직학형태화면역표형특점장해조병변분위삼충모식。모식1：4례，형태학상유사우CHL，단종류세포CD20미만강양성；모식2：8례，형태학상유사우DLBCL，단종류세포이질성표체CD20、PAX5，동시표체CD30화/혹CD15；모식3：4례，형태특점개우CHL화DLBCL지간，종류세포불동정도지표체CD20、CD30화CD15。11례수검병례중，6례종류세포표체EB병독잠복막단백1。림상상，절대다수환자대연합리타석단항화CHOP（ R-CHOP）방안적면역화료불민감。결론통과묘술삼충상견적조직학모식，제출료구유DLBCL화CHL중간특점적회구림파류적진단표준。저류종류외주형병변화종격병변적병리특점사유불동。목전대저조교계성종류상무유효치료방법，환자예후교차。
Objective To profile the clinicopathologic features of a series of grey zone lymphoma (GZL) cases with hybrid features of diffuse large B-cell lymphoma ( DLBCL) and classical Hodgkin lymphoma (CHL), with a purpose to gain an in-depth understanding of the borderline B-cell neoplasm. Methods The clinical, morphologic and immunophenotyical characteristics of 16 cases were retrospectively analyzed.Results The patients were mostly male adults , with a male to female ratio of 1.7∶1.0 and a mean age of 40.2 years.Eight patients presented with peripheral nodal lesions and five cases with mediastinal involvement .Histologically and immunophenotypically , the 16 cases were classified into three sub-categories.In 4 cases, the morphologic features resembled CHL more closely , but the neoplastic cells showed uniform and intense positive staining of CD 20 ( pattern 1 ) .Although the initial impression of the other 8 cases was that of DLBCL , the expression levels of CD 20 and PAX5 were variable , and CD30 or CD15 was positive (pattern 2).A characteristic feature of pattern 3, observed in the remaining 4 cases, demonstrated a broad spectrum of morphology with hybrid features of both CHL and DLBCL .The neoplastic cells in pattern 3 were positive for CD20, CD30 and CD15.EBV-LMP1 was detected in 6 of the 11 tested cases.Clinically, most patients with GZL seemed insensitive to immuno-chemotherapy of the R-CHOP regimen.Conclusions The diagnostic criteria for GZL with features intermediate between DLBCL and CHL is proposed by the three histologic patterns commonly seen in these lesions .Cases presented with peripheral lesions might differ from those with mediastinal presentation pathologically .At current time , there is no effective treatment for these borderline B-cell lymphomas and the prognosis is poor .