Grey zone lymphoma with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma: a...

To investigate the clinicopathological features of Epstein-Barr virus (EBV)-positive grey zone lymphoma (GZL) with features intermediate between diffuse large B-cell lymphoma (DLBCL) and classical Hodgkin lymphoma (CHL).

We investigated the clinicopathological features of 14 cases of EBV-positive GZL in Japan. The control group included 173 cases of EBV-positive CHL and 64 cases of EBV-positive DLBCL of the elderly (polymorphous type). The patients were 10 men and four women with a median age of 62 years. Twelve patients (86%) had advanced clinical stage, 11 (79%) had B-symptoms, eight (57%) had mediastinal disease, 10 (71%) had elevated serum lactate dehydrogenase (LDH) levels, and five (36%) had thrombocytopenia. All cases had CHL-like morphology but strongly expressed at least one B-cell marker. The neoplastic cells were Hodgkin and Reed-Sternberg-like cells, but with a large number of mononuclear variants. EBV-positive GZL patients were more significantly more likely than EBV-positive CHL patients to have advanced clinical stage (P = 0.023), presence of B-symptoms (P = 0.011), elevated serum LDH levels (P = 0.047), thrombocytopenia (P = 0.042), and mediastinal involvement (P = 0.023). The progression-free survival (PFS) of EBV-positive GZL patients was significantly poorer than that of EBV-positive CHL patients (P = 0.043) but no difference from EBV-positive DLBCL patients was observed (P = 0.367).

EBV-positive GZL patients have significantly worse PFS than EBV-positive CHL patients, and are significantly more likely to have adverse clinical parameters such as advanced clinical stage, presence of B-symptoms, and thrombocytopenia. Further studies are needed to better characterize this entity, which may require the development of innovative therapeutic strategies.