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Official websites use. Share sensitive information only on official, secure websites. Large granular lymphocyte LGL leukemia is a T or NK clonal disorder characterized by the tissue invasion of marrow, spleen and liver. Clinical presentation is dominated by recurrent infections associated with neutropenia, anemia, splenomegaly and autoimmune diseases, particularly rheumatoid arthritis.
Indications for treatment are severe or symptomatic neutropenia, symptomatic or transfusion-dependent anemia or associated autoimmune diseases requiring therapy. There is no standard treatment for patients with LGL leukemia. All the largest series published in the literature collecting data on more than 40 patients are retrospective. Data are very heterogeneous and treatment outcome per single agent is available for very few patients.
Immunosuppressive therapy remains the foundation of treatment including single agents that is, methotrexate, oral cyclophosphamide or cyclosporine. On the basis of an initial study showing very good overall response rate ORR using methotrexate, this drug has remained the most recommended option in LGL leukemia. In this letter, we describe the encouraging results of cyclophosphamide used in a series of 45 previously untreated LGL leukemia patients.
Patients suffering from LGL leukemia and treated with cyclophosphamide as first-line therapy were included in this retrospective study.
Patients gave their informed consent for data collection. Treatment failure was defined as a progressive disease worsening of cytopenia or organomegaly or a stable disease none of the later given criteria met.