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

Andrew M. Evens, Jennifer A. Kanakry, Laurie H. Sehn, Athena Kritharis, Tatyana Feldman, Aimee Kroll, Randy D. Gascoyne, Jeremy S. Abramson, Adam M. Petrich, Francisco J. Hernandez-Ilizaliturri, Zeina Al-Mansour, Camille Adeimy, Jessica Hemminger, Nancy L. Bartlett, Anthony Mato, Paolo F. Caimi, Ranjana H. Advani, Andreas K. Klein, Chadi Nabhan, Sonali M. SmithJesus C. Fabregas, Izidore S. Lossos, Oliver W. Press, Timothy S. Fenske, Jonathan W. Friedberg, Julie M. Vose, Kristie A. Blum

Research output: Contribution to journalArticlepeer-review

36 Scopus 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.

Original languageEnglish (US)
Pages (from-to)778-783
Number of pages6
JournalAmerican Journal of Hematology
Volume90
Issue number9
DOIs
StatePublished - Sep 1 2015

ASJC Scopus subject areas

  • Hematology

Fingerprint Dive into the research topics of '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'. Together they form a unique fingerprint.

Cite this