Concurrent Epstein-Barr virus associated non-Hodgkin lymphoma and recurrent Hodgkin disease

Purpose: Patients treated for Hodgkin disease (HD) are at increased risk for developing secondary neoplasms; including non-Hodgkin lymphoma (NHL). We present a patient who developed NHL (brain and lungs) as a second malignancy together with recurrent HD (bone marrow). Because HD and NHL have both been associated with Epstein-Barr virus (EBV), especially in the immunodeficient host, the tumor specimens were studied for the presence of EBV, and the possible role of immunodeficiency in the development of this second malignancy was assessed. Methods and Results: Tumor specimens were analyzed by conventional histologic and immunohistochemical methods, EBV detection was determined by RNA in situ hybridization for EBV-encoded RNAs (EBERs). Histopathology showed diffuse large cell lymphoma of B-cell phenotype in specimens from lesions in the brain and lungs. These specimens were EBER+. HD specimens from all recurrences were evaluated and were EBER-. Conclusions: This patient had received extensive chemoradiotherapy for recurrent HD, and presented with Pneumocystis pneumonia, a low absolute T-cell count, no response to mitogens in vitro, a second malignancy (EBV + NHL), and recurrent EBVHD. We propose that the immunocompromised state of the patient played a significant role in the development of his second malignancy.