Discordant Lymphoma: A Sequential Case of Burkitt Lymphoma and Classic Hodgkin Lymphoma

Abstract

Abstract Casestudy A discordant lymphoma is a rare condition in which two or more subtypes of lymphomas can occur in different anatomic sites; the distinct sub-classifications can present concurrently or sequentially. We present a rare case of a second primary Classic Hodgkin Lymphoma (CHL) several years after achieving remission from an unusual presentation of a non-Hodgkin lymphoma (NHL). Results A 40-year-old HIV-positive male presented to the emergency department for acute cholecystitis. Sections of his gallbladder revealed atypical monomorphic lymphocyte infiltrate that were diffusely positive for CD10, CD20, BCL6, and PAX5, and negative for BCL2, CD5, Cyclin D1, and MUM1. The proliferation index by Ki-67 was 100%. EBER ISH is positive. A FISH assay for the C-MYC break apart probe and dual fusion of IgH and BCL2 showed a rearrangement of 8q24, highly suspicions for a 8;14 translocation, classically seen in Burkitt Lymphoma (BL). Subsequently, he was placed on RA-DA-EPOCH and achieved remission. Seven years later, he presented with nausea, vomiting, headaches, and blurry vision. An excisional biopsy of his right cervical lymph node was performed. The histological sections showed effaced nodal architecture with sclerotic fibrosis and necrosis. There were scattered Hodgkin cells/Reed-Sternberg (HRS) cells in the background of lymphocytes, plasma cells, histiocytes, and eosinophils. The HRS cells were positive for CD15, CD20 (weak), CD30, BCL6, MUM1, and PAX5 (weak), and negative for ALK, CD10, and CD45. EBER ISH was positive and HHV8 was negative. These features were consistent with a CHL, nodular sclerosis subtype. Conclusion Malignancies in HIV patients pose a unique challenge as they often possess distinct characteristics distinct, including unique aspects of tumor localization, atypical pathological features, unusual growth behavior, and advanced stage at presentation. Epstein-Barr virus (EBV) is strongly linked with several HIV-associated lymphomas, as it encodes several proteins including LMP-1, LMP-2A, BHRF-1, EBNA-3A, and EBNA-3C that are implicated in the development of NHLs. The identification of discordant lymphomas may have variable prognoses and different treatment modalities. In addition, the study of such discordant lymphoma cases may provide more insight on the etiology and inter-relationship of clonal evolution in lymphoma.