Abstract
Primary hepatic lymphoma (PHL) is an extremely rare disease, frequently associated with viruses such as hepatitis B virus (HBV), hepatitis C virus (HCV), and human immune deficiency virus (HIV). On the other hand, an increased risk of lymphoproliferative disorders (LPD) has been demonstrated in patients treated with immunosuppressive drugs such as methotrexate (MTX) for rheumatoid arthritis (RA). The role of Epstein-Barr virus (EBV) has been discussed in the pathogenesis of the immunodeficiency-associated LPDs. We here describe a RA patient, who developed PHL during RA treatment. The patient was a 64 year-old Japanese male with a 2-year history of RA, who had been treated with MTX at weekly dose of 8–14 mg for 2 years and infliximab (IFX) for 7 months. He presented with a 2 month history of generalized malaise, right hypochondrium pain and fever. Contrast-enhanced computed tomography (CECT) of the abdomen showed multiple irregular and nodular liver masses with a maximum of 13 cm in diameter on the right liver. Biopsy specimens demonstrated CD20-positve diffuse large B-cell lymphoma (DLBCL), but EBV was not identified by EBV-encoded RNA in situ hybridization. Serology for HBV, HCV, human T-cell leukemia virus I (HTLV-I), and HIV was negative. His symptoms disappeared following discontinuation of RA treatment including MTX. A drastic regression of the tumor masses was further obtained without cytotoxic chemotherapy. In addition, although the patient had no past history of liver dysfunction before MTX therapy, persistent elevation of liver enzymes has been observed during MTX treatment. These findings show a causative role of MTX in the development of reversible PHL in the patient.
Highlights
Lymphoproliferative disorders (LPDs) developed in autoimmune disease patients receiving immunosuppressive therapy are categorized as other iatrogenic immunodeficiency-associated lymphoproliferative disorders (LPD) in the WHO classification of tumors of hematopoietic and lymphoid tissues [1]
There has been no significant history of liver dysfunction before MTX therapy, persistent elevation of liver enzymes has been observed during MTX treatment; aspartate aminotransferase (AST) 36 to 128 U/L and alanine aminotransferase (ALT) 44 to 93 U/L
The findings were consistent with non-Hodgkin lymphoma (NHL), diffuse large B-cell lymphoma (DLBCL), and according to the WHO 2008 classification of lymphoid tissue, he was diagnosed as other iatrogenic immunodeficiency-associated LPDs
Summary
Lymphoproliferative disorders (LPDs) developed in autoimmune disease patients receiving immunosuppressive therapy are categorized as other iatrogenic immunodeficiency-associated LPD in the WHO classification of tumors of hematopoietic and lymphoid tissues [1]. Case presentation A 64-year-old man presented with a 2 month history of generalized malaise, right hypochondrium pain and fever. He had a history for appendectomy at the age of 42 and sarcoidosis and RA at the age of 62. The findings were consistent with non-Hodgkin lymphoma (NHL), diffuse large B-cell lymphoma (DLBCL), and according to the WHO 2008 classification of lymphoid tissue, he was diagnosed as other iatrogenic immunodeficiency-associated LPDs. RA treatment including MTX was discontinued. He became asymptomatic, and serum LDH returned to normal levels. There has been no sign of recurrence for two years
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