Pathogenesis of neutropenia in large granular lymphocyte leukemia and Felty syndrome.

摘要: T-cell large granular lymphocyte leukemia (TLGL) is an atypical chronic lymphoproliferative disorder derived from cytotoxic T-cells (CTL). Unlike most forms of leukemia, the pattern bone marrow infiltration in TLGL may be subtle and cytopenias are often lineage specific, with neutropenia dominating. Both granulocytic survival proliferation defects observed mediated by humoral cell-mediated mechanisms respectively. Splenic production immune complexes induces a neutrophil defect, where as Fas expression leukemic CTL results based defect. These pathways induce apoptosis through independent intracellular which not mutually exclusive concurrently individual patients either or FS. A variety therapeutic interventions have been utilized management Felty syndrome, including methotrexate, cyclosporine A, cyclophosphamide, glucocorticoids, myeloid colony stimulating factors splenectomy. Their efficacy action reviewed.