B-lymphoproliferative disorders: a proposed unified pathogenetic pathway.
The clinical features of lymphoproliferative diseases associated with paraproteinemia are briefly reviewed and correlated with current immunologic concepts in an effort to clarify the pathophysiology of B-lymphocyte disorders. B-lymphocyte maturation proceeds in a predictable manner from the Pre-B cell to the formation of idiotype specific plasma cells and memory B-lymphocytes. The immunoglobulin isotype produced by the mature plasma cell is determined by a site specific process of gene switching which proceeds from mu to alpha production. Lymphoproliferative diseases are the result of disordered B cell maturation and their clinical features can be explained by identifying the locus of the maturational defect.
D000328:Adult; D000916:Antibody Diversity; D001402:B-Lymphocytes / Q000276:immunology* / Q000473:pathology; D002454:Cell Differentiation; D005260:Female; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications / Q000276:immunology / Q000473:pathology; D007070:Immunoglobulin A / Q000032:analysis; D007074:Immunoglobulin G / Q000032:analysis; D007075:Immunoglobulin M / Q000032:analysis; D007156:Immunologic Memory; D007945:Leukemia, Lymphoid / Q000150:complications / Q000276:immunology / Q000473:pathology; D016403:Lymphoma, Large B-Cell, Diffuse / Q000150:complications / Q000276:immunology / Q000473:pathology; D008228:Lymphoma, Non-Hodgkin / Q000150:complications / Q000276:immunology / Q000473:pathology; D008232:Lymphoproliferative Disorders / Q000150:complications / Q000276:immunology* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009101:Multiple Myeloma / Q000150:complications / Q000276:immunology / Q000473:pathology; D008258:Waldenstrom Macroglobulinemia / Q000150:complications / Q000276:immunology / Q000473:pathology
D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007075:Immunoglobulin M