Clinicopathologic, immunophenotypic, cytogenetic, and molecular features of γδ T-Cell large granular lymphocytic leukemia: An analysis of 14 patients suggests biologic differences with γδ T-Cell large granular lymphocytic Leukemia

Mariko Yabe, L. Jeffrey Medeiros, Sa A. Wang, Sergej Konoplev, Chi Young Ok, Sanam Loghavi, Gary Lu, Lauren Flores, Joseph D. Khoury, R. Craig Cason, Ken H. Young, Roberto N. Miranda

Research output: Contribution to journalArticlepeer-review

30 Scopus citations

Abstract

Objectives: T-cell large granular lymphocytic (T-LGL) leukemia is a rare disorder in which the neoplastic cells usually express the αβ T-cell receptor (TCR). To determine the significance of γδ TCR expression in this leukemia, we compared the clinicopathologic, immunophenotypic, and genetic features of patients with T-LGL leukemia expressing γδ TCR or αβ TCR. Methods: We used the World Health Organization classification criteria to confirm the diagnosis. All patients were diagnosed and treated at our institution. Results: We identified 14 patients with γδ T-LGL leukemia, 11 men and three women; six (43%) patients had a history of rheumatoid arthritis, 10 (71%) had neutropenia, four (29%) had thrombocytopenia, and three (21%) had anemia. Eight (67%) of 12 patients had a CD4-/CD8-phenotype, and four (33%) had a CD4-/CD8+ phenotype. The median overall survival was 62 months. Patients with γδ T-LGL leukemia were more likely to have rheumatoid arthritis (P = .04), lower absolute neutrophil count (P = .04), lower platelet count (P = .004), and a higher frequency of the CD4-/CD8-phenotype (P < .0001). However, there was no significant difference in overall survival between the two groups (P = .64). Conclusions: Although patients with γδ and αβ T-LGL leukemia show some different clinical or phenotypic features, overall survival is similar, suggesting that γδ TCR expression does not carry prognostic significance.

Original languageEnglish (US)
Pages (from-to)607-619
Number of pages13
JournalAmerican journal of clinical pathology
Volume144
Issue number4
DOIs
StatePublished - Oct 2015

Keywords

  • Gd T-cell receptor
  • Hepatosplenic T-cell lymphoma
  • Rheumatoid arthritis
  • T-cell large granular lymphocytic leukemia

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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