Gray Zone Lymphoma: Current Diagnosis and Treatment Options

Monika Pilichowska; Athena Kritharis; Andrew M Evens

doi:10.1016/j.hoc.2016.07.006

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.

Authors

Monika Pilichowska¹, Athena Kritharis², Andrew M Evens³

Affiliations

¹ Department of Pathology and Laboratory Medicine, Tufts Medical Center, 800 Washington Street, Boston, MA 02111, USA.
² Division of Hematology/Oncology, Tufts Medical Center, 800 Washington Street, Boston, MA 02111, USA.
³ Division of Hematology/Oncology, Tufts Medical Center, 800 Washington Street, Boston, MA 02111, USA. Electronic address: AEvens@tuftsmedicalcenter.org.

PMID: 27888879
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