A fulminant case of classical Hodgkin lymphoma: A diagnostic dilemma of Epstein‐Barr virus‐positive large B‐cell neoplasms

Abstract

We report a fulminant case of classical Hodgkin lymphoma (CHL). The patient died only approximately 2 months after the onset of subjective symptoms. Autopsy specimens revealed atypical cells resembling Hodgkin and Reed‐Sternberg (HRS) cells in a rich inflammatory background in various organs. There were marked, characteristic angiodestructive lesions from infiltrating HRS‐like cells and numerous macrophages. The HRS‐like cells were infected with Epstein‐Barr virus (EBV), immunohistochemically positive for PAX5 and CD30, and negative for CD3, CD20, and ALK. Most B‐cell markers other than PAX5 were negative, and the HRS‐like cells also expressed cytotoxic molecules. Monoclonal rearrangement of immunoglobulin heavy chain was detected by PCR analysis. According to the 2016 WHO classification, we diagnosed mixed cellularity CHL. However, EBV‐positive diffuse large B‐cell lymphoma (DLBCL), not otherwise specified and EBV‐positive B‐cell lymphoma, unclassifiable with features intermediate between DLBCL and CHL were considered as differential diagnoses because both tumors are aggressive EBV‐positive large B‐cell neoplasms with reactive inflammatory cells and sometimes contains HRS‐like cells. The clinical condition of the current case was closer to these two entities than to CHL. A diagnosis of EBV‐positive large B‐cell neoplasms was difficult because of overlapping morphological and immunohistochemical characteristics, but should be considered for prognosis.