Iatrogenic immunodeficiency-associated lymphoproliferative disease of the Hodgkin lymphoma-like variant in a patient treated with mycophenolate mofetil for autoimmune hepatitis

Immunodeficiency-associated lymphoproliferative disorders (ILDs) are well-described entities that occur in the setting of organ transplant, bone marrow transplant, and congenital or acquired immunodeficiency [1,2]. The latest World Health Organization (WHO) classification of tumors of hematopoietic and lymphoid tissues [3] defines a subset of other iatrogenic ILDs, composed mostly of lymphoproliferations occurring in the setting of patients receiving immunosuppressive therapy (particularly methotrexate or anti-TNF (tumor necrosis factor) agents) for autoimmune diseases or conditions other than in the transplant setting such as rheumatoid arthritis [4-9] or inflammatory bowel disease [10]. The role of Epstein-Barr virus (EBV) in ILDs is well characterized and the EBV-positive ILDs have a better prognosis than their EBV negative counterparts [9]. Among the morphologic variants of iatrogenic ILDs are classical Hodgkin lymphoma (cHL) and Hodgkin-like lymphoproliferations resembling cHL. These are rare entities with very few cases reported in the medical literature [4,8,11,12]. We report a case of EBV-positive ILD of the Hodgkin-like variant in a patient on long-term mycophenolate mofetil (MMF) for autoimmune hepatitis. Our patient had a complete response after withdrawal of immunosuppression and treatment with rituximab. As many of these cases will resolve without cytotoxic chemotherapy, this case highlights the importance of recognizing this clinical entity [4] and distinguishing it from de novo lymphomas.