Clinicopathologic, Immunophenotypic, Cytogenetic, and Molecular Features of γδ T-Cell Large Granular Lymphocytic Leukemia: An Analysis of 14 Patients Suggests Biologic Differences With αβ T-Cell Large Granular Lymphocytic Leukemia. [corrected].

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.

• Authors: 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
• Journal: American journal of clinical pathology (Am J Clin Pathol) Vol. 144 Issue 4 Pg. 607-19 (Oct 2015) ISSN: 1943-7722 [Electronic] England
• PMID: 26386082 (Publication Type: Journal Article)
• Copyright: Copyright© by the American Society for Clinical Pathology.

Chemical References

• Receptors, Antigen, T-Cell, alpha-beta
• Receptors, Antigen, T-Cell, gamma-delta

Topics

• Adult
• Aged
• Aged, 80 and over
• Arthritis, Rheumatoid (epidemiology, etiology)
• Female
• Flow Cytometry
• Humans
• Immunohistochemistry
• Immunophenotyping
• Kaplan-Meier Estimate
• Leukemia, Large Granular Lymphocytic (immunology, mortality, pathology)
• Male
• Middle Aged
• Polymerase Chain Reaction
• Receptors, Antigen, T-Cell, alpha-beta
• Receptors, Antigen, T-Cell, gamma-delta (immunology)
• Young Adult