Reclassification of diffuse large B cell lymphoma to large B cell lymphoma with IRF4 rearrangement in an adult population

Author:

Hesius Eva A M1,van Laar Lidia23,Oosterveld Margriet4,van Spriel Annemiek B5,Scheijen Blanca6,Leeuwis Jan Willem2,Marres Henri A M7,Groenen Patricia J T A6,Stevens Wendy B C1,van der Spek Ellen8,van den Brule Adriaan J C9,Hoevenaars Brigiet M10,Hebeda Konnie M6,van den Brand Michiel26ORCID

Affiliation:

1. Department of Hematology Radboud University Medical Center Nijmegen the Netherlands

2. Pathology‐DNA Rijnstate Hospital Arnhem the Netherlands

3. Pathology‐DNA, St Antonius Hospital Nieuwegein the Netherlands

4. Department of Hematology Canisius‐Wilhelmina Hospital Nijmegen the Netherlands

5. Department of Tumor Immunology Radboud Institute for Molecular Life Sciences, Radboud University Medical Center Nijmegen the Netherlands

6. Department of Pathology Radboud University Medical Center Nijmegen the Netherlands

7. Department of Otorhinolaryngology and Head and Neck Surgery Radboud University Medical Center Nijmegen the Netherlands

8. Department of Hematology Rijnstate Hospital Arnhem the Netherlands

9. Pathology‐DNA Jeroen Bosch Hospital Den Bosch the Netherlands

10. Department of Pathology Canisius‐Wilhelmina Hospital Nijmegen the Netherlands

Abstract

AimsLarge 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 fluorescence in‐situ hybridisation (FISH) for IRF4.Methods and resultsWith FISH, we studied the presence of IRF4 rearrangements in 238 lymphomas that were diagnosed as DLBCL according to the previous WHO classification of 2008.ConclusionsIn addition to the index patient, an IRF4 rearrangement was detected in another five of 237 patients (2%). The immunohistochemical profile of these five IRF4 rearranged lymphomas was consistent with previous reports of LBCL‐IRF4. One case was recognised to represent transformation of follicular lymphoma rather than de‐novo LBCL‐IRF4. BCL6 rearrangements were found in two 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.

Funder

European Research Council

KWF Kankerbestrijding

Publisher

Wiley

Subject

General Medicine,Histology,Pathology and Forensic Medicine

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