We presented a 64-year-old male patient with T-large granular cell leukemia/lymphoma with an agressive clinical course. Large granular lymphocytes were noted on peripheral blood smear. The phenotyping of the cells was typical T-cell lineage [CD2 (+), CD3 (+), CD5 (+)]. Clonal rearrangement of the T-cell receptor gene (TCR) was demonstrated by DNA hybridization technique. Large granular cell leukemia/lymphoma is a distinct entity with spesific clinicobiological aspects. The clinical spectrum is wide and immunophenotyping and genotyping studies need to make a diagnosis.
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