Epstein-Barr virus positive anaplastic large cell lymphoma: myth or reality?

The World Health Organization (WHO) Classification of Tumours of Haematopoietic and Lymphoid Tissue, 2008 edition, states that anaplastic large cell lymphoma (ALCL) is "consistently negative for Epstein-Barr virus (EBV)". The statement made by the WHO has led to the widespread belief that EBV can have no pathogenic role in ALCL. Herein we report a case of an immunocompetent 35-year-old male who presented with hemophagocytic syndrome secondary to lymphoma for which diagnostic material consisted solely of a bone marrow biopsy. The biopsy demonstrated large anaplastic cells which were uniformly positive for surface CD3, CD30 (strong membranous and Golgi expression), CD45, TIA-1 and Granzyme B but negative for ALK-1. In-situ hybridization was strongly positive for EBER in the large neoplastic cells. The uniformity of CD30 expression and positivity for cytotoxic markers on the anaplastic tumor cells raised the diagnostic possibility of an EBV-associated ALCL, ALK-. Discussion of this case as well as a retrospective review of 64 cases of reported of EBV+ ALCL are presented.