Faculty Collaboration Database

Gray zone lymphoma with features intermediate between classical Hodgkin lymphoma and diffuse large B-cell lymphoma: characteristics, outcomes, and prognostication among a large multicenter cohort. Am J Hematol 2015 Sep;90(9):778-83

Date

06/06/2015

Pubmed ID

26044261

DOI

10.1002/ajh.24082

Scopus ID

2-s2.0-84939529582 (requires institutional sign-in at Scopus site)   72 Citations

Abstract

Gray zone lymphoma (GZL) with features between classical Hodgkin lymphoma and diffuse large B-cell lymphoma (DLBCL) is a recently recognized entity reported to present primarily with mediastinal disease (MGZL). We examined detailed clinical features, outcomes, and prognostic factors among 112 GZL patients recently treated across 19 North American centers. Forty-three percent of patients presented with MGZL, whereas 57% had non-MGZL (NMGZL). NMGZL patients were older (50 versus 37 years, P = 0.0001); more often had bone marrow involvement (19% versus 0%, P = 0.001); >1 extranodal site (27% versus 8%, P = 0.014); and advanced stage disease (81% versus 13%, P = 0.0001); but they had less bulk (8% versus 44%, P = 0.0001), compared with MGZL patients. Common frontline treatments were cyclophosphamide-doxorubicin-vincristine-prednisone +/- rituximab (CHOP+/-R) 46%, doxorubicin-bleomycin-vinblastine-dacarbazine +/- rituximab (ABVD+/-R) 30%, and dose-adjusted etoposide-doxorubicin-cyclophosphamide-vincristine-prednisone-rituximab (DA-EPOCH-R) 10%. Overall and complete response rates for all patients were 71% and 59%, respectively; 33% had primary refractory disease. At 31-month median follow-up, 2-year progression-free survival (PFS) and overall survival rates were 40% and 88%, respectively. Interestingly, outcomes in MGZL patients seemed similar compared with that of NMGZL patients. On multivariable analyses, performance status and stage were highly prognostic for survival for all patients. Additionally, patients treated with ABVD+/-R had markedly inferior 2-year PFS (22% versus 52%, P = 0.03) compared with DLBCL-directed therapy (CHOP+/-R and DA-EPOCH-R), which persisted on Cox regression (hazard ratio, 1.88; 95% confidence interval, 1.03-3.83; P = 0.04). Furthermore, rituximab was associated with improved PFS on multivariable analyses (hazard ratio, 0.35; 95% confidence interval, 0.18-0.69; P = 0.002). Collectively, GZL is a heterogeneous and likely more common entity and often with nonmediastinal presentation, whereas outcomes seem superior when treated with a rituximab-based, DLBCL-specific regimen.

Author List

Evens AM, Kanakry JA, Sehn LH, Kritharis A, Feldman T, Kroll A, Gascoyne RD, Abramson JS, Petrich AM, Hernandez-Ilizaliturri FJ, Al-Mansour Z, Adeimy C, Hemminger J, Bartlett NL, Mato A, Caimi PF, Advani RH, Klein AK, Nabhan C, Smith SM, Fabregas JC, Lossos IS, Press OW, Fenske TS, Friedberg JW, Vose JM, Blum KA

Author

Timothy Fenske MD Professor in the Medicine department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Adult
Aged
Antibodies, Monoclonal, Murine-Derived
Antineoplastic Combined Chemotherapy Protocols
Bone Marrow
Cyclophosphamide
Doxorubicin
Drug Administration Schedule
Etoposide
Female
Hodgkin Disease
Humans
Lymphoma, Large B-Cell, Diffuse
Male
Mediastinum
Middle Aged
Multivariate Analysis
Neoplasm Staging
Prednisone
Prognosis
Retrospective Studies
Rituximab
Survival Analysis
Vincristine