HODGKINS-DISEASE-LIKE LYMPHOPROLIFERATIVE DISORDERS IN PATIENTS WITH DIFFERENT UNDERLYING IMMUNODEFICIENCY STATES

Epstein-Barr virus (EBV)-associated lymphoproliferative diseases are a frequent occurrence in immnnodeficient patients. Most commonly seen a re polymorphic B-cell lymphoproliferative disorders and malignant B-ce ll lymphomas, Classical Hodgkin's disease (HD), however, is rare in th ese patients. In the present study, we attempted to characterize cases resembling HD in patients with a variety of underlying immunodeficien cy states using clinical aspects, immunohistochemistry, in situ hybrid ization, and polymerase chain reaction, All of the six cases that we i nvestigated presented clinically with B symptoms and a short, devastat ing course. Histologically, they showed a lymphocytic depletion and mu ltiple cells that resembled HD and Reed-Sternberg cells. Many of those were atypical blast cells that stained positively for B-cell surface antigens, such as CD20 and CD79a, whereas others were of the typical H D and Reed-Sternberg phenotype, Another frequent finding, especially i n the extranodal sites, was a perivascular arrangement of large blast cells intermingled with small lymphoid cells, All of the cases were EB V-associated (proved latent membrane protein-1 immunohistochemical ana lysis, EBV-encoded RNA irt situ hybridization, and polymerase chain re action for subtypes A and B), with a frequent coinfection of type A an d B. This was in contrast to ordinary HD, which is characterized by si ngle infection of only one strain, i.e., the subtype A in Western coun tries. Three cases showed clonal B-cell populations, at least at termi nal stages of the disease, We describe a lymphoproliferative disorder in immunodeficient patients with features of HD that, in some respects , resembles an EBV-driven lymphoproliferative disorder seen in cases o f fatal infectious mononucleosis. We conclude that clinical and pathol ogic features of these disorders might cause some problems for histolo gic differential diagnosis and might represent a separate entity of th eir own in immunodeficient patients.