Aberrant GATA3 expression in Large B-cell Lymphoma with IRF4 rearrangement: a pitfall in the pathological diagnosis, a case report

Abstract Background Large B-cell lymphoma (LBCL) with interferon regulatory factor 4 (IRF4) rearrangement (LBCL-IRF4) is a rare entity of LBCL with a specific clinical presentation. Notably, it is defined by the presence of an IRF4 rearrangement. Case presentationHere, we describe a case of a 2-years-old child presenting with a LBCL-IRF4 located in the left tonsil, with characteristic histologic appearance and the phenotype of neoplastic cells. The histologic analysis showed the monomorphic atypical lymphoid cells were medium size or large, positive for CD20, CD79a, MUM-1, GATA3, and Ki-67 was about 80%; Fluorescence in situ hybridization (FISH) analysis confirmed the presence of an IRF4 rearrangement. The patient was followed up for 18 months with no evidence of recurrence after being treated with 1 cycles of R-CHOP.ConclusionMorphologically, LBCL-IRF4 has predominantly diffuse or follicular pattern with a large number of necrosis, and a high proliferation rate, all of these suggest that the tumor is highly invasive. But actually LBCL-IRF4 usually performs a favorable outcome after therapy. Furthermore, LBCL-IRF4 with GATA3 positive have never been reported.