Sistema gastrointestinal y digestivo

Abstracto

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

Minesh Mehta, Jiang Wang, Ross McHenry, Stephen Zucker

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.