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Abstract：
Large B-cell lymphoma with IRF4 rearrangement, and Burkitt-like lymphoma with 11q aberration are two provisional lymphoma entities in the 2017 revision of the WHO classification of lymphoid neoplasms. Despite being more frequent in young patients, knowledge regarding their true incidence and clinical features in unselected cohorts of paediatric and adolescent patients is limited. We screened for both entities among paediatric patients (<18 years of age) in the German NHL-BFM (Non-Hodgkin lymphoma Berlin-Frankfurt-Münster) group. Among follicular lymphomas and diffuse large B-cell lymphomas (DLBCL), 7/34 cases (21%) showed an IRF4 break-apart pattern by fluorescence in situ hybridisation (FISH) and are associated with stages I and II disease (P = 0·043). Among lymphomas morphologically resembling Burkitt lymphoma, DLBCL and high-grade B-cell lymphoma, unclassifiable, 13/102 cases (13%) lacked a MYC break-apart pattern but were positive for 11q proximal gain and telomeric loss by FISH. MYC-negative Burkitt-like lymphomas with the typical 11q gain-loss pattern by FISH were older (P = 0·004), showed less male predominance (P = 0·003), lower stage (P = 0·040), lower serum LDH level (P = 0·01) and less abdominal involvement (P = 0·008) compared to high grade B-cell lymphomas without 11q gain-loss pattern. Both entities showed excellent outcome with overall survival of 100% when managed according to NHL-BFM strategies and may provide candidates for future therapy de-escalation in clinical trials.
摘要：
大B细胞淋巴瘤伴IRF4重排,和具有11q畸变的Burkitt样淋巴瘤是2017年WHO淋巴样肿瘤分类修订版中的两个临时淋巴瘤实体。尽管年轻患者更频繁，在未选择的儿科和青少年患者队列中，有关其真实发病率和临床特征的知识有限。我们在德国NHL-BFM（柏林-法兰克福-明斯特非霍奇金淋巴瘤）组的儿科患者（<18岁）中筛查了这两种实体。在滤泡性淋巴瘤和弥漫性大B细胞淋巴瘤（DLBCL）中，7/34例（21％）通过荧光原位杂交（FISH）显示IRF4断裂模式，并与I期和II期疾病相关（P=0·043）。在形态上类似于伯基特淋巴瘤的淋巴瘤中，DLBCL和高级别B细胞淋巴瘤，不可分类，13/102例（13％）缺乏MYC分裂模式，但通过FISH对11q近端增益和端粒丢失呈阳性。MYC阴性的Burkitt样淋巴瘤具有典型的11q增益-损失模式，年龄较大（P=0·004），男性优势较少(P=0.003)，较低阶段(P=0·040)，与没有11q增益-损失模式的高级别B细胞淋巴瘤相比，血清LDH水平较低（P=0·01）和腹部受累较少（P=0·008）。当根据NHL-BFM策略进行管理时，两个实体均显示出优异的结果，总生存率为100％，并且可能为临床试验中的未来治疗降级提供候选人。
