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2-Deoxycoformycin in the treatment of T-large granular lymphocyte leukemia

Authors
Journal
Leukemia Research
0145-2126
Publisher
Elsevier
Publication Date
Volume
27
Issue
9
Identifiers
DOI: 10.1016/s0145-2126(03)00019-5
Keywords
  • T-Lgl
  • Neutropenia
  • 2-Deoxycoformycin
  • Pentostatin
  • Vβ Repertoire
Disciplines
  • Medicine

Abstract

Abstract A 52-year-old woman presented to our clinic for investigation of agranulocytosis and mild lymphocytosis. A diagnosis of T-large granular lymphocyte leukemia was made, based on immunophenotyping findings of the peripheral blood lymphocytes (CD3, CD8, CD16, CD57). Flow cytometric analysis of TCR-Vβ repertoire showed single Vβ9 expression on peripheral T-cells. Clonality was also demonstrated with PCR analysis which revealed clonal rearrangement of TCRγ-chain gene. Granulocyte-macrophage colony-stimulating factor (G-CSF) (G-CSF), cyclosporine, methylprednisolone and oral methotrexate failed to correct the neutropenia. Finally, treatment with 2-deoxycoformycin (DCF) was successful and resulted in complete correction of the neutrophil count. Flow cytometric analysis of TCR-Vβ repertoire proved to be an effective method to assess the therapeutic response to various treatments and to evaluate residual disease.

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