2-deoxycoformycin in the treatment of T-large granular lymphocyte leukemia.
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-Vbeta repertoire showed single Vbeta9 expression on peripheral T-cells. Clonality was also demonstrated with PCR analysis which revealed clonal rearrangement of TCRgamma-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-Vbeta repertoire proved to be an effective method to assess the therapeutic response to various treatments and to evaluate residual disease.
Tsirigotis
Panagiotis
P
Third Department of Internal Medicine, First Hospital of Athens, Social Insurance Foundation, Zaimi Street-Melissia, 15127 Athens, Greece. tsirsi1@otenet.gr
Venetis
Evangelos
E
Kapsimali
Violeta
V
Rontogianni
Dimitra
D
Varvitsioti
Ephrosini
E
Pappa
Vasiliki
V
Apostolidis
Panos
P
D000903:Antibiotics, Antineoplastic / Q000627:therapeutic use*; D015703:Antigens, CD / Q000378:metabolism; D005260:Female; D005434:Flow Cytometry; D019674:Genes, T-Cell Receptor beta / Q000502:physiology; D006801:Humans; D007135:Immunoglobulin Variable Region / Q000276:immunology*; D016130:Immunophenotyping; D007945:Leukemia, Lymphoid / Q000188:drug therapy* / Q000235:genetics / Q000276:immunology; D015458:Leukemia, T-Cell / Q000188:drug therapy* / Q000235:genetics / Q000276:immunology; D008875:Middle Aged; D009503:Neutropenia / Q000097:blood / Q000150:complications / Q000188:drug therapy; D015649:Pentostatin / Q000627:therapeutic use*; D012074:Remission Induction
D000903:Antibiotics, Antineoplastic; D015703:Antigens, CD; D007135:Immunoglobulin Variable Region; D015649:Pentostatin