Large Granular Lymphocyte Leukemia论文.docVIP

　　Large Granular Lymphocyte Leukemia论文 .freelir Sokola, Thomas P. Loughran, Jr.b 【关键词】 T-cell,LGL,leukemia, #8226;,Aggressive,NK-cell,leukemia, #8226;,Cytopenia, #8226;,Autoimmunity LEARNING OBJECTIVES After pleting this course,.freelolecular and cellular biology of LGL leukemia. Describe distinct clinical entities among disorders of LGLs. Discuss the diagnostic criteria for T-cell LGL leukemia. Discuss the therapeutic algorithm of LGL leukemia. ABSTRACT Clonal disorders of large granular lymphocytes (LGLs) represent a spectrum of biologically distinct lymphoproliferative diseases originating either from mature T cells (CD3+) or natural killer (NK) cells (CD3). Both subtypes, T-cell and NK-cell LGL leukemia, can manifest as indolent or aggressive disorders. The majority of patients ia have a clinically indolent course edian survival time 10 years. Immunosuppressive therapy ethotrexate, cyclophosphamide, or cyclosporine A can control symptoms and cytopenias in more than 50% of patients, but this approach is not curative. Several cases of an aggressive variant (CD3+CD56+) of T-cell LGL leukemia ia is usually a rapidly progressive disorder associated erica. This disease is usually refractory to conventional chemotherapy, edian survival time of 2 months. Chronic NK-cell leukemia/lymphocytosis is a rare EBV-negative disorder alignant origin of this subtype is uncertain because clonality is difficult to determine in LGLs of NK-cell origin. DEFINITION AND CLASSIFICATION Large granular lymphocytes (LGLs) represent 10%15% of the total peripheral blood mononuclear cells in normal adults 1. The majority of these cells (85%) are derived from the CD3 natural killer (NK)cell lineage, and a minority are derived from the CD3+ T-cell lineage (15%). Cytologically, LGLs are medium to large cells ic, antigen-primed cytotoxic CD8+ T lymphocytes. NK-cell LGLs belong to the innate immune system ajor histopatibility plex (MHC)restricted cytotoxicity. LGL leukemia arros