title: T-cell large granular lymphocyte leukemia associated with myelodysplastic syndrome: a clinicopathologic study of nine cases.
abstract: We describe 9 patients with T-cell large granular lymphocyte leukemia (T-LGL) who also had a myelodysplastic syndrome (MDS). There were 6 men and 3 women with a median age of 60 years (range, 25-74 years). All patients had anemia at initial examination, 7 had neutropenia, and 5 had thrombocytopenia. The median absolute lymphocyte count was 1,300/microL (1.3 x 10(9)/L; range, 700-3,600/microL [0.7-3.6 x 10(9)/L]). Immunophenotypic analysis showed a CD8+ T-cell population, and molecular analysis showed monoclonal T-cell receptor gene rearrangement in every case. The MDS was classified as refractory cytopenia with multilineage dysplasia (RCMD, n = 5), refractory anemia (n = 2), RCMD with ringed sideroblasts (n = 1), and chronic myelomonocytic leukemia (n = 1). We compared the data for these patients with T-LGL/MDS with a group that had only T-LGL. The median hemoglobin level and absolute lymphocyte count were lower in patients with T-LGL/MDS (P < .05). The frequency of coexistent T-LGL and MDS at our institution suggests an etiologic relationship rather than simple coincidence.
authors.affiliation: Department of Hematopathology, Unit 72, University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA.
authors.forename: Yang O
authors.initials: YO
authors.lastname: Huh
authors.forename: L Jeffrey
authors.initials: LJ
authors.lastname: Medeiros
authors.forename: Farhad
authors.initials: F
authors.lastname: Ravandi
authors.forename: Sergej
authors.initials: S
authors.lastname: Konoplev
authors.forename: Jeffrey L
authors.initials: JL
authors.lastname: Jorgensen
authors.forename: Roberto N
authors.initials: RN
authors.lastname: Miranda
mesh_terms: D000328:Adult; D000368:Aged; D015139:Blotting, Southern; D005260:Female; D005434:Flow Cytometry; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; D006801:Humans; D016130:Immunophenotyping; D054066:Leukemia, Large Granular Lymphocytic / Q000150:complications* / Q000235:genetics* / Q000473:pathology*; D018655:Lymphocyte Count; D008297:Male; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000150:complications* / Q000235:genetics* / Q000473:pathology*; D016133:Polymerase Chain Reaction