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Refining the diagnosis of T-cell large granular lymphocytic leukemia by combining distinct patterns of antigen expression with T-cell clonality studies.

Abstract
T-cell large granular lymphocytic (LGL) leukemia is a complex diagnosis, requiring persistent clonal expansions of LGLs, and cytopenias. Often the diagnosis is unclear as non-clonal expansions of LGLs commonly occur in reactive conditions. To better understand T-LGL leukemia, we performed a comprehensive clinicopathologic analysis of 85 patients with LGL expansions. Interestingly, distinct CD8+(dim)/CD57+ populations, seen by flow cytometry, were significantly associated with clonal T-LGL leukemia (P < 0.001) as well as neutropenia (median absolute neutrophil count (ANC) 1.45 vs 3.19 × 10(9)/l; P = 0.0017). Furthermore, cases with distinct CD8+(dim)/CD57+ populations and monoclonal T cells had even lower ANCs (median ANC 1.41 × 10(9)/l; P = 0.001) compared with cases without these dual criteria. Additionally, complete or partial loss of CD5 expression was independently associated with clonal T-LGL leukemia (P<0.001) and neutropenia (median ANC 1.41 vs 2.70 × 10(9)/l; P = 0.002). This study describes specific immunophenotypic parameters to better define clonal cases of T-LGL leukemia associated with significant neutropenia.
AuthorsR S Ohgami, J K Ohgami, I T Pereira, G Gitana, J L Zehnder, D A Arber
JournalLeukemia (Leukemia) Vol. 25 Issue 9 Pg. 1439-43 (Sep 2011) ISSN: 1476-5551 [Electronic] England
PMID21617700 (Publication Type: Journal Article)
Chemical References
  • Receptors, Antigen, T-Cell, alpha-beta
Topics
  • Adult
  • Aged
  • Aged, 80 and over
  • Clone Cells
  • Female
  • Flow Cytometry
  • Gene Rearrangement, beta-Chain T-Cell Antigen Receptor (genetics, immunology)
  • Humans
  • Immune System (immunology)
  • Immunophenotyping
  • Inflammation (diagnosis, immunology, metabolism)
  • Leukemia, Large Granular Lymphocytic (diagnosis, immunology, metabolism)
  • Male
  • Middle Aged
  • Neutropenia (diagnosis, immunology, metabolism)
  • Receptors, Antigen, T-Cell, alpha-beta (genetics, metabolism)
  • T-Lymphocytes (immunology, metabolism)
  • Young Adult

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