Gray Zone Lymphoma: Current Diagnosis and Treatment Options

Hematol Oncol Clin North Am. 2016 Dec;30(6):1251-1260. doi: 10.1016/j.hoc.2016.07.006.

Abstract

The morphology of gray zone lymphoma (GZL) is variable with tumor cells spanning the spectrum of diffuse large B-cell lymphoma (DLBCL) and classical Hodgkin lymphoma. The immunophenotype is frequently discordant. Clinically, the initial case descriptions of GZL were primarily with mediastinal presentation; however, a nonmediastinal (systemic) clinical subtype is now recognized. Patients with GZL have high relapse rates. Recommended treatment of GZL is with a DLBCL-directed regimen with consideration for consolidative radiotherapy for bulk disease. Continued biologic examination of this entity is needed and there should be exploration toward integration of novel targeted therapeutic agents into the treatment paradigm of GZL.

Keywords: B-cell; Biology; Classical Hodgkin lymphoma; Diffuse large B-cell lymphoma; Gray zone lymphoma; Prognosis; Treatment.

Publication types

  • Review

MeSH terms

  • Chemoradiotherapy / methods*
  • Consolidation Chemotherapy / methods*
  • Hodgkin Disease / diagnosis*
  • Hodgkin Disease / pathology
  • Hodgkin Disease / therapy*
  • Lymphoma, Large B-Cell, Diffuse / diagnosis*
  • Lymphoma, Large B-Cell, Diffuse / pathology
  • Lymphoma, Large B-Cell, Diffuse / therapy*
  • Recurrence