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.

中文翻译：

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在NHL-BFM组的儿科患者中，对具有IRF4重排的临时WHO实体大B细胞淋巴瘤和11q畸变的Burkitt样淋巴瘤的经验。

2017年修订的WHO淋巴肿瘤分类中，具有IRF4重排的大型B细胞淋巴瘤和具有11q畸变的Burkitt样淋巴瘤是两个临时淋巴瘤实体。尽管在年轻患者中发病率更高，但对于未选择的小儿和青少年患者队列，有关其真实发病率和临床特征的知识仍然有限。我们在德国NHL-BFM（非霍奇金淋巴瘤柏林-法兰克福-明斯特）组中的儿科患者（年龄小于18岁）中对这两个实体进行了筛查。在滤泡性淋巴瘤和弥漫性大B细胞淋巴瘤（DLBCL）中，有7/34例病例（21％）通过荧光原位杂交（FISH）显示IRF4断裂模式，并与I和II期疾病相关（P = 0·043）。在形态学上类似于Burkitt淋巴瘤，DLBCL和高级别B细胞淋巴瘤的淋巴瘤中，无法分类的13/102例（13％）缺乏MYC分离模式，但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FISH的典型11q增减型，年龄较大（P  = 0·004），男性优势较少（P  = 0·003），晚期（P  = 0·040），较低血清LDH水平（P  = 0·01）和较少的腹部受累（P = 0·008）与没有11q增减型的高级别B细胞淋巴瘤相比。根据NHL-BFM策略进行管理时，这两个实体均显示出优异的结局，总体生存率为100％，并可能为临床试验中未来治疗的降级提供候选。