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. 2023 Aug 9;483(3):281–298. doi: 10.1007/s00428-023-03590-x

Box 1

• HG/LBCL-11q is defined by chromosome 11q-gains and telomeric loss and can occur in the setting of immunodeficiency including post-transplant and HIV+ patients but also in patients with ataxia-telangiectasia.

• FISH analysis is recommended for the diagnosis of HG/LBCL-11q.

• LMO2 is a useful diagnostic marker for HG/LBCL-11q but expressed only in 50% of the cases. Burkitt lymphoma is LMO2 negative.

• LBCL-IRF4 is mainly a disease of children and young adults but it also occurs in adults and elderly patients.

• LBCL-IRF4 in children and young adults affects predominantly the Waldeyer’s ring and presents in clinical stages I/II; in contrast, in adults, it is more often a nodal disease and presents in advanced clinical stages (III/IV).

• LBCL-IRF4 is an indolent lymphoma with excellent prognosis.

• Morphologically, LBCL-IRF4 can be purely follicular, follicular/diffuse, or diffuse and often shows aberrant expression of CD10, BCL6, and MUM1. CD5 expression is not rare, especially in children.

• In adults, IRF4 rearrangements can be observed in a variety of aggressive B-cell lymphomas in association with other chromosomal alterations. These cases should not be diagnosed as LBCL-IRF4.

• Other novel molecular groups are discussed.