T-Cell/Histiocyte-Rich Large B-Cell Lymphoma Masquerading as Autoimmune Hepatitis with Clinical Features of Hemophagocytic Lymphohistiocytosis

Abstract

We report the case of a 20-year-old male who presented with acute hepatitis resembling autoimmune hepatitis, but subsequently found to be T-Cell Rich/Histiocyte Rich Large B-Cell Lymphoma. Our patient was mistakenly diagnosed with autoimmune hepatitis based on liver histology demonstrating a pronounced lobular and portal infiltrate comprised predominately of polyclonal T cells, in the setting of negative serologic testing. This conclusion was reinforced by a compelling biochemical response to standard immunosuppressive therapy. The correct diagnosis of T/HRBCL subsequently was established by bone marrow biopsy (and confirmed by lymph node biopsy) when the patient presented with clinical features of HLH. In conclusion, our case elucidates a unique clinical spectrum of T/HRBCL. This patient initially presented with acute hepatitis, before mistakenly being diagnosed with autoimmune hepatitis and finally exhibited clinical features of HLH, which led to the diagnosis of T/HRBCL. It is critical to consider lymphoma in the differential for acute hepatopathy and clinical features of HLH.

Unique presentation of T-cell/histiocyte-rich large B cell lymphoma complicated with hemophagocytic lymphohistiocytosis: Case report and review of the literature

“…To the best of our knowledge, eight cases of HLH secondary to TCHRLBCL were described in the English literature (19)(20)(21)(22)(23)(24)(25)(26). Their summary is displayed in Table II.…”