Aims
Large B-cell lymphoma with IRF4 rearrangement (LBCL-IRF4) is a new entity in the 2017 revised World Health Organisation (WHO) Classification, that was initially mainly reported in children. After identification of a 79-year-old patient we assessed how often IRF4 rearrangements can be detected in adult diffuse large B-cell lymphomas (DLBCLs) which have to be reclassified to LBCL-IRF4 based on fluorescent in-situ hybridisation (FISH) for IRF4.
Methods and results
With FISH, we studied the presence of IRF4 rearrangements in 238 lymphomas that were diagnosed as DLBCL according to the previous WHO classification of 2008.
Conclusions
In addition to the index patient, an IRF4 rearrangement was detected in another 5 out of 237 patients (2%). The immunohistochemical profile of these five IRF4 rearranged lymphomas was consistent with previous reports of LBCL-IRF4. One case was recognized to represent transformation of follicular lymphoma rather than de novo LBCL-IRF4. BCL6 rearrangements were found in 2 cases of LBCL-IRF4, BCL2 and MYC rearrangements were excluded. Patients presented with limited stage disease with involvement of the head and neck in three patients, and involvement of the lung and thyroid in two others. This study shows that, although rare, LBCL-IRF4 should also be considered in older patients and at localisations other than the head and neck region.
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