title: Grey zone lymphoma with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma: a clinicopathological study of 14 Epstein-Barr virus-positive cases.
abstract: OBJECTIVE
  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).
  
  
  RESULTS
  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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Pathology and Laboratory Medicine, Nagoya University Hospital, Nagoya, Japan.|Department of Pathology, Faculty of Medicine, Mansoura University, Mansoura, Egypt.
authors.forename: Ahmed A
authors.initials: AA
authors.lastname: Elsayed
authors.affiliation: Department of Pathology and Laboratory Medicine, Nagoya University Hospital, Nagoya, Japan.
authors.forename: Akira
authors.initials: A
authors.lastname: Satou
authors.affiliation: Department of Pathology and Laboratory Medicine, Nagoya University Hospital, Nagoya, Japan.|Department of Pathology, Faculty of Medicine, Mansoura University, Mansoura, Egypt.
authors.forename: Ahmed E
authors.initials: AE
authors.lastname: Eladl
authors.affiliation: Department of Pathology and Laboratory Medicine, Nagoya University Hospital, Nagoya, Japan.
authors.forename: Seiichi
authors.initials: S
authors.lastname: Kato
authors.affiliation: Department of Pathology and Laboratory Medicine, Nagoya University Hospital, Nagoya, Japan.
authors.forename: Shigeo
authors.initials: S
authors.lastname: Nakamura
authors.affiliation: Department of Clinical Laboratory, Nagano Prefectural Suzaka Hospital, Nagano, Japan.
authors.forename: Naoko
authors.initials: N
authors.lastname: Asano
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000032:analysis; D018572:Disease-Free Survival; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D005260:Female; D006689:Hodgkin Disease / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D017403:In Situ Hybridization; D007564:Japan; D053208:Kaplan-Meier Estimate; D008223:Lymphoma / Q000401:mortality / Q000473:pathology* / Q000821:virology; D016403:Lymphoma, Large B-Cell, Diffuse / Q000473:pathology; D008297:Male; D008875:Middle Aged
chemical_list: D014408:Biomarkers, Tumor
keywords: Epstein-Barr virus; classical Hodgkin lymphoma; diffuse large B-cell lymphoma; grey zone lymphoma