title: 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: OBJECTIVE
  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.affiliation: From the Departments of Hematopathology and.
authors.forename: Mariko
authors.initials: M
authors.lastname: Yabe
authors.affiliation: From the Departments of Hematopathology and.
authors.forename: L Jeffrey
authors.initials: LJ
authors.lastname: Medeiros
authors.affiliation: From the Departments of Hematopathology and.
authors.forename: Sa A
authors.initials: SA
authors.lastname: Wang
authors.affiliation: From the Departments of Hematopathology and.
authors.forename: Sergej
authors.initials: S
authors.lastname: Konoplev
authors.affiliation: From the Departments of Hematopathology and.
authors.forename: Chi Young
authors.initials: CY
authors.lastname: Ok
authors.affiliation: From the Departments of Hematopathology and.
authors.forename: Sanam
authors.initials: S
authors.lastname: Loghavi
authors.affiliation: From the Departments of Hematopathology and.
authors.forename: Gary
authors.initials: G
authors.lastname: Lu
authors.affiliation: Translational Molecular Pathology, The University of Texas MD Anderson Cancer Center, Houston.
authors.forename: Lauren
authors.initials: L
authors.lastname: Flores
authors.affiliation: From the Departments of Hematopathology and.
authors.forename: Joseph D
authors.initials: JD
authors.lastname: Khoury
authors.affiliation: From the Departments of Hematopathology and.
authors.forename: R Craig
authors.initials: RC
authors.lastname: Cason
authors.affiliation: From the Departments of Hematopathology and.
authors.forename: Ken H
authors.initials: KH
authors.lastname: Young
authors.affiliation: From the Departments of Hematopathology and Roberto.miranda@mdanderson.org.
authors.forename: Roberto N
authors.initials: RN
authors.lastname: Miranda
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001172:Arthritis, Rheumatoid / Q000453:epidemiology / Q000209:etiology; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D053208:Kaplan-Meier Estimate; D054066:Leukemia, Large Granular Lymphocytic / Q000276:immunology* / Q000401:mortality / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D016133:Polymerase Chain Reaction; D016693:Receptors, Antigen, T-Cell, alpha-beta; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000276:immunology*; D055815:Young Adult
chemical_list: D016693:Receptors, Antigen, T-Cell, alpha-beta; D016692:Receptors, Antigen, T-Cell, gamma-delta
keywords: Hepatosplenic T-cell lymphoma; Rheumatoid arthritis; T-cell large granular lymphocytic leukemia; γδ T-cell receptor