T-cell large granular lymphocytic leukaemia: successful response to 2-deoxycoformycin.
We report a 25-year-old woman with T-cell large granular lymphocytic leukaemia presenting with severe neutropenia, anaemia and recurrent infections with a chronic disease course. Immunophenotyping showed an expansion of CD3+, TCRgamma delta+, CD4-, CD5+, CD7+, CD8+, CD57+ large granular lymphocytes. Clonality was demonstrated with T-gamma polymerase chain reaction analysis which revealed clonal rearrangement of the TCRgamma chain gene. Cyclosporine, granulocyte colony-stimulating factor, methothrexate and a combination of cyclophosphamide, vincristine and prednisolone failed to correct the neutropenia and the anaemia. Finally, treatment with 2-deoxycoformycin resulted in both clinical and haemotological complete responses, despite molecular evidence of the persistence of the abnormal T-cell clone.
Dincol
G
G
Istanbul University, Istanbul Faculty of Medicine, Department of Internal Medicine, Division of Haematology, Capa, Istanbul, Turkey.
Diz-Kuçukkaya
R
R
Bicakci
E
E
D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D054066:Leukemia, Large Granular Lymphocytic / Q000188:drug therapy*; D015649:Pentostatin / Q000627:therapeutic use*
D000970:Antineoplastic Agents; D015649:Pentostatin