Neutropenia in a Patient With Rheumatoid Arthritis Associated With alpha-beta/gamma-delta T-Cell Large Granular Lymphocyte Lymphoproliferative Disorder Manifesting as Felty’s Syndrome: From Diagnosis to Therapy

Abstract

T-cell large granular lymphocytic (LGL) leukaemias are clonal proliferations of cytotoxic T-cells that are frequently associated with rheumatoid arthritis (RA) and neutropenia and are usually controlled with immunosuppressive drugs and growth factors. We report a case of a 51-years-old patient with RA, mild splenomegaly and severe autoimmune neutropenia, who have failed to respond to methotrexate, leflunamide and corticosteroids and had a suspicion of granulocyte-colony stimulating factor (G-CSF) induced thrombocytopenia. The diagnosis of Felty's syndrome was done and phenotypically abnormal TCR-αβ and TCRγδ T-LGL were detected in blood, although PCR-based studies provided evidence only for clonal TCRG gene rearrangements. A second attempt to control neutropenia with G-CSF induced thrombocytopenia again. Cyclosporine A was effective in controlling neutropenia, the dose being adjusted in order to maintain acceptable neutrophil counts and to minimise side effects. These included hypertension, renal insufficiency and gingival hypertrophy, the later being worsened by the concomitant use of amlodipine.