Large granular lymphocytic leukemia (LGLL) is a rare clonal lymphoproliferative disorder from T or NK origin.
Purpose: to report on the diagnostic and therapeutic management of LGLL investigated in the university hospital at Nancy, France.
Methods: retrospective (7 years) collection of clinical and biological data and patients' cohort analysis.
Results: Eight out of fifteen patients presented with neutropenia, including five profound neutropenia (neutrophils < 500 × 109/L). Four patients had an infection. Two patients have rheumatoid arthritis and an associated Felty's syndrome, one a Sweet syndrome. Two also suffered from chronic Lymphocytic Leukemia, and one from a diffuse large B-cell lymphoma. Twelve patients had LGLL-T and 3 had a chronic LGLL-NK. Eleven out of twelve patients had a clonal LGLL-T when polymerase chain reaction assessed. No KIR clonality was sought among the 3 LGL-NK patients. Five patients out of fifteen received immunosuppressive treatment.
Conclusion: Although using simple and robust investigations, our series demonstrates a high heterogeneity in LGLL detection and assessment.
Keywords: clonality; diagnosis; immunophenotyping; large granular lymphocyte leukemia.