Experience with provisional WHO‐entities large B‐cell lymphoma with IRF4‐rearrangement and Burkitt‐like lymphoma with 11q aberration in paediatric patients of the NHL‐BFM group

Abstract

Summary 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.

A Case of Burkitt-Like Lymphoma With 11q Aberration With HIV Infection in East Asia and Literature Review

“…Despite the poor prognostic factors in our patient, such as advancedstage disease, BM involvement, and low absolute lymphocyte count, low-intensity chemotherapy (DA-EPOCH-R) was effective. These outcomes strengthen the favorable prognosis of BLL-11q and suggest consideration of dose-reduced therapy for this subgroup of lymphomas [3,10].…”

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Dear Editor, Burkitt-like lymphoma with 11q aberration (BLL-11q) is a new provisional classification in the revised fourth edition of WHO classification of lymphomas that resembles Burkitt lymphoma (BL) morphologically and phenotypically, but has unique features, including gains in 11q23.2-23.3 and losses of 11q24.1qter, with no MYC translocation [1]. BLL-11q predominantly occurs in young adult males, and characteristic cytogenetic features have been frequently observed in post-transplant patients [2][3][4]. However, the association between BLL-11q and immune deficiency remains unclear due to the limited number of cases [2,[4][5][6].

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Aggressive B-cell lymphoma cases with 11q aberration patterns indicate a spectrum beyond Burkitt-like lymphoma

“…From a clinical perspective, the course of BLL-11q has only been systematically reported in pediatric patients, where it was shown to compare favorably to other aggressive lymphomas 21 . In contrast, Sevilla et al reported MYC-negative Burkitt-like-lymphoma, prior to the discovery of the 11q-gain/loss-pattern, to harbor an inferior outcome, compared with MYCpositive BL 22 .…”

Molecular Update and Evolving Classification of Large B-Cell Lymphoma

“…The presence of IRF4 rearrangements identified by FISH studies, together with BCL6 rearrangements and a uniform lack of BCL2 and MYC rearrangements, are the defining features of LBCL-IRF4 [116,120,124]. Some cases may additionally exhibit IRF4/IGH fusions but are negative for IRF4 rearrangement using commercially available break-apart probes [125]. The involvement of light chain genes has been rarely reported [116].…”