large granular lymphocyte leukemia.docVIP

Large Granular Lymphocyte Leukemia nbsp;nbsp;nbsp;nbsp;nbsp;nbsp;nbsp;nbsp;nbsp;nbsp;nbsp;nbsp; 作者：Lubomir Sokola, Thomas P. Loughran, Jr.b 【关键词】nbsp; T-cell,LGL,leukemia,amp;#8226;,Aggressive,NK-cell,leukemia,amp;#8226;,Cytopenia,amp;#8226;,Autoimmunity 　　LEARNING OBJECTIVESnbsp; 　　After completing this course, the reader will be able to: 　　Discuss the basic principles of molecular 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. 　　ABSTRACTnbsp; nbsp; 　　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 with T-cell LGL leukemia have a clinically indolent course with a median survival time gt;10 years. Immunosuppressive therapy with low-dose methotrexate, 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 with a poor prognosis have also been reported. Aggressive NK-cell LGL leukemia is usually a rapidly progressive disorder associated with Epstein-Barr virus (EBV), with a higher prevalence in Asia and South America. This disease is usually refractory to conventional chemotherapy, with a median survival time of 2 months. Chronic NK-cell leukemia/lymphocytosis is a rare EBV-negative disorder with an indolent clinical course. The malignant origin of this subtype is uncertain because clonality is difficult to determine in LGLs of NK-cell origin. 　　DEFINITION AND CLASSIFICATIONnbsp; nbsp; 　　Large granular lymphocytes (LGLs