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

Tsirigotis, Panagiotis; Venetis, Evangelos; Kapsimali, Violeta; Rontogianni, Dimitra; Varvitsioti, Ephrosini; Pappa, Vasiliki; Apostolidis, Panos

doi:10.1016/S0145-2126(03)00019-5

« Previous Next »Leukemia Research
Volume 27, Issue 9 , Pages 865-867, September 2003

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

Panagiotis Tsirigotis
- Third Department of Internal Medicine, First Hospital of Athens, Social Insurance Foundation, Zaimi Street-Melissia, 15127 Athens, Greece
- Corresponding author. Tel.: +30-1-8042320; fax: +30-1-8041837.
Evangelos Venetis
- Third Department of Internal Medicine, First Hospital of Athens, Social Insurance Foundation, Zaimi Street-Melissia, 15127 Athens, Greece
Violeta Kapsimali
- Immunology-Histocompatibility Department, Evangelismos Hospital, Athens, Greece
Dimitra Rontogianni
- Histopathology Department, Evangelismos Hospital, Athens, Greece
Ephrosini Varvitsioti
- Third Department of Internal Medicine, First Hospital of Athens, Social Insurance Foundation, Zaimi Street-Melissia, 15127 Athens, Greece
Vasiliki Pappa
- Second Department of Internal Medicine, Evangelismos Hospital, Athens, Greece
Panos Apostolidis
- Third Department of Internal Medicine, First Hospital of Athens, Social Insurance Foundation, Zaimi Street-Melissia, 15127 Athens, Greece

Received 14 October 2002; accepted 10 January 2003.

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.

Keywords: T-LGL, Neutropenia, 2-Deoxycoformycin, Pentostatin, Vβ repertoire