Lymphoma and Pathology Committee, JCCG (Japan Children's Cancer Group)/JPLSG (Japan Pediatric Leukemia/Lymphoma Study Group), Tokyo, Japan.
Department of Hematology/Oncology for Children and Adolescents, Sapporo Hokuyu Hospital, Sapporo, Hokkaido, Japan.
Pediatr Blood Cancer. 2023 May;70(5):e30279. doi: 10.1002/pbc.30279. Epub 2023 Mar 1.
Diffuse large B-cell lymphoma (DLBCL) is classified into two molecular subtypes according to its cell of origin: germinal center B-cell (GCB) subtype and activated B-cell/non-GCB subtype. This latter subtype shows a poorer prognosis in adults. However, in pediatric DLBCL, the prognostic impact of the subtype is yet to be clarified.
This study sought to compare the prognosis between GCB and non-GCB DLBCL in a large number of cases in children and adolescents. In addition, this study intended to describe the clinical, immunohistochemical, and cytogenetic characteristics of these two molecular subtypes of DLBCL, and consider differences in the biology, frequency, and prognosis of GCB and non-GCB subtypes in pediatric versus adult DLBCL or in Japanese versus Western pediatric DLBCL patients.
DESIGN/METHODS: We selected mature B-cell lymphoma/leukemia patients for whom specimens had been submitted to the central pathology review in Japan between June 2005 and November 2019. We referred the past studies on Asian adult patients and Western pediatric patients to compare with our results.
Data were obtained from 199 DLBCL patients. The median age of all patients was 10 years, with 125 patients (62.8%) in the GCB group and 49 (24.6%) in the non-GCB group other than 25 cases whose immunohistochemical data were insufficient. Overall, the percentage of translocation of MYC (1.4%) and BCL6 (6.3%) was lower than in adult and Western pediatric DLBCL cases. The non-GCB group showed a significantly higher proportion of females (44.9%), a higher incidence of stage III disease (38.8%), and B-cell lymphoma 2 (BCL2)-positivity in immunohistochemistry (79.6%) compared to the GCB group; however, no BCL2 rearrangement was observed in both GCB and non-GCB groups. The prognosis did not differ significantly between the GCB and non-GCB groups.
This study including a large number of non-GCB patients showed the same prognosis between GCB and non-GCB groups and suggested a difference in the biology of pediatric and adolescent DLBCL compared to adult DLBCL as well as between Asian and Western DLBCL.
弥漫性大 B 细胞淋巴瘤 (DLBCL) 根据其细胞起源可分为两个分子亚型：生发中心 B 细胞 (GCB) 亚型和活化 B 细胞/非 GCB 亚型。后一种亚型在成人中的预后较差。然而，在儿科 DLBCL 中，该亚型的预后影响尚不清楚。
本研究旨在比较大量儿童和青少年 GCB 和非 GCB DLBCL 患者的预后。此外，本研究旨在描述这两种 DLBCL 分子亚型的临床、免疫组织化学和细胞遗传学特征，并考虑 GCB 和非 GCB 亚型在儿科与成人 DLBCL 或日本与西方儿科 DLBCL 患者中的生物学、频率和预后差异。
设计/方法：我们选择了 2005 年 6 月至 2019 年 11 月间日本中央病理审查提交标本的成熟 B 细胞淋巴瘤/白血病患者。我们参考了亚洲成人患者和西方儿科患者的既往研究结果，与我们的结果进行了比较。
从 199 例 DLBCL 患者中获得数据。所有患者的中位年龄为 10 岁，其中 125 例（62.8%）为 GCB 组，49 例（24.6%）为非 GCB 组，另有 25 例免疫组织化学数据不足。总体而言，MYC（1.4%）和 BCL6（6.3%）易位的比例低于成人和西方儿科 DLBCL 病例。与 GCB 组相比，非 GCB 组女性比例（44.9%）较高，III 期疾病发生率（38.8%）较高，免疫组化中 B 细胞淋巴瘤 2（BCL2）阳性率（79.6%）较高；然而，在 GCB 和非 GCB 组均未观察到 BCL2 重排。GCB 和非 GCB 组之间的预后无显著差异。
本研究包括大量非 GCB 患者，结果表明 GCB 和非 GCB 组之间的预后相同，并表明儿科和青少年 DLBCL 的生物学与成人 DLBCL 以及亚洲和西方 DLBCL 之间存在差异。
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