Nakagawa A, Nakamura S, Ito M, Shiota M, Mori S, Suchi T
Department of Pathology, Nagoya University Hospital, Japan.
Mod Pathol. 1997 Mar;10(3):210-5.
CD30(+)-anaplastic large cell lymphoma (ALCL) is a distinct clinicopathologic entity of non-Hodgkin's lymphoma that is immunologically heterogeneous. Bimodal age distribution, a nonrandom chromosome abnormality [t(2;5)(p23;q35)] that produces a chimeric protein p80npm/alk, and a variable (5-47%) association with Epstein-Barr virus (EBV) have been reported. We reviewed 36 cases of ALCL (19 were children < 20 yr and 17 were adults) by focusing on the presence or absence of p80npm/alk protein and EBV. Immunophenotyping studies were performed on frozen and/or paraffin sections before initiation of chemotherapy. The p80 protein was immunohistochemically examined, and EBV-encoded RNA transcripts were detected by in situ hybridization. Among 19 cases in children, 13 cases had a T-cell and 6 cases had a null-cell phenotype. p80 Was detected in 16 (84.2%) of 19 cases in children and 3 (17.6%) of 17 cases in adults. All of the 19 cases of p80-positive ALCL were positive for epithelial membrane antigen, regardless of age. EBV genome was not detected in any of 19 cases in children and in only 2 of 15 cases in adults. ALCL in childhood seems to constitute a homogeneous entity characterized by expression of p80 and absence of EBV. The association of EBV is also infrequent in adult ALCL among Japanese patients.
CD30(+)间变性大细胞淋巴瘤（ALCL）是一种独特的非霍奇金淋巴瘤临床病理实体，在免疫方面具有异质性。据报道，其年龄分布呈双峰型，存在一种产生嵌合蛋白p80npm/alk的非随机染色体异常[t(2;5)(p23;q35)]，并且与爱泼斯坦-巴尔病毒（EBV）有可变（5 - 47%）关联。我们通过关注p80npm/alk蛋白和EBV的有无，对36例ALCL病例（19例为20岁以下儿童，17例为成人）进行了回顾。在化疗开始前，对冰冻和/或石蜡切片进行免疫表型研究。采用免疫组织化学方法检测p80蛋白，通过原位杂交检测EBV编码的RNA转录本。19例儿童病例中，13例为T细胞表型，6例为空细胞表型。19例儿童病例中有16例（84.2%）检测到p80，17例成人病例中有3例（17.6%）检测到p80。19例p80阳性的ALCL病例，无论年龄大小，上皮膜抗原均为阳性。19例儿童病例中均未检测到EBV基因组，15例成人病例中仅2例检测到。儿童期ALCL似乎构成一个以p80表达和EBV缺失为特征的同质实体。在日本患者的成人ALCL中，EBV的关联也不常见。
