Large granular lymphocyte leukemia. A rare disease with personalized treatment options

Abstract

Introduction: Large granular lymphocyte leukemia is rare, mainly chronic disease. The most common complication is neutropenia, but other immune-mediated cytopenia may also occur. There are no unified treatment recommendations and initiation of treatment mainly depends on the severity of the symptoms. Aim: The aim of the authors was to analyze the main steps of the diagnosis and the necessity and outcome of treatment in their patients diagnosed with large granular lymphocyte leukaemia. Method: The authors retrospectively analyzed the data of 17 large granular lymphocyte leukemia patients. Results: Of the 17 patients, 7 patients required treatment because of transfusion dependent anemia (4 patients) or neutropenia (3 patients). In 4 patients corticosteroid was given (supplemented with cyclosporine in one patients), while the other patients received anti-CD52 (one patient), low dose methotrexate (one patient) and combined chemotherapy (one patient). Five patients achieved partial response, and two patients died in sepsis. Conclusions: In this cohort only a smaller proportion of patients required therapy. Immunosuppression can be successful, but the effect in most cases was temporary. The most serious complication was sepsis, which is associated with a significant risk of mortality in cases with neutropenia. Orv. Hetil., 2014, 155(11), 414–419.