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Large granular lymphocytic leukemia (LGLL) is a rare clonal lymphoproliferative disorder of T or NK origin. Objective: To report on the diagnostic and therapeutic management of LGLL investigated at Nancy University Hospital, France. Method: Retrospective (7 years) collection of clinical and biological data and patients’ cohort analysis. Results: Eight out of fifteen patients presented with neutropenia, five of whom had profound neutropenia (neutrophils < 500 × 109/L). Four patients had an infection. Two patients had rheumatoid arthritis, one of whom also had Felty’s and the other 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 12 patients had a clonal LGLL-T when polymerase chain reaction was assessed. No KIR clonality was sought among the 3 LGL-NK patients. Five patients out of 15 received immunosuppressive treatment. Conclusion: Although it used simple and robust investigations, our case series demonstrates a high heterogeneity in LGLL detection and assessment.