Case Report: Composite Angioimmunoblastic T-Cell Lymphoma and Epstein-Barr Virus-Positive B-Cell Lymphoproliferative Disorder as Other Iatrogenic Immunodeficiency-Associated Lymphoproliferative Disorders

Abstract

Immunosuppressants are widely used to treat patients with rheumatoid arthritis (RA), and their adverse effects have been known to cause other iatrogenic immunodeficiency-associated lymphoproliferative disorders (OIIA-LPDs). We report a patient with RA who had been treated with methotrexate (MTX) and tacrolimus (TAC) and who developed whole body lymphadenopathy. We simultaneously confirmed angioimmunoblastic T-cell lymphoma (AITL) through a right cervical lymph node biopsy and Epstein-Barr virus-positive B-cell lymphoproliferative disorder (EBV-positive B-LPD) through a bone marrow examination. After cessation of immunosuppressant therapy, both LPDs completely disappeared. Patients with AITL are occasionally reported to develop B-cell lymphoma through reactivation of the EBV, which leads to clonal expansion in the microenvironment. Immunohistochemistry results revealed that both LPD components were positive for EBV-encoded RNA. Moreover, in this patient, the plasma EBV DNA level was found to be high; therefore, EBV infection was a probable etiology. Synchronous coexistence of AITL and B-LPD as an OIIA-LPD has rarely been reported. This case report is the first to discuss the disappearance of both LPDs on withdrawal of immunosuppressants only. AITL occasionally accompany B-LPD; however, this composite lymphoma comprised AITL and B-LPD, and OIIA-LPDs should not be overlooked.

Composite diffuse large B-cell lymphoma and peripheral T-cell lymphoma: a case report with two-year follow-up and literature review

“…Alternatively, the first appearance of T-cell lymphoma may also result in a deficiency in innate immunity that renders host B cells more susceptible to EBV infection, leading to transformation into B-cell neoplasms. In such cases, the patient’s T-cell and B-cell tumor components tend to be EBER positive ( 8 , 21 ). Although this patient was positive for EBV-associated IgG, the pathology indicated that EBER was negative for both T and B cell components.…”

Epstein–Barr Virus-Positive Langerhans Cell Sarcoma: Is There a Link? A Case Report

“…Moreover, immunosuppressants, including methotrexate (MTX) and tacrolimus (TAC), are widely used to treat patients with rheumatoid arthritis (RA), and their adverse effects have been known to cause other iatrogenic immunodeficiency-associated lymphoproliferative disorders (OIIA-LPDs). Seiji reported that the tumor cells were positive for EBV in 8 (17%) of 48 patients; background cells were positive in 32 (82%) of 39 patients with available data in the literature review of MTX-associated T-LPDs (MTX T-LPDs) ( 68 ). The presence of EBV reflects a profound immunodeficiency and may drive the development or a rapid progression of the tumor.…”