[Physiopathology of monoclonal IgM associated with peripheral neuropathy].
Monoclonal IgM associated with a peripheral neuropathy often feature an antibody activity directed against the myelin-associated-glycoprotein. The main characteristics of the structure of these IgM are reviewed: the variable gene repertoire used by these antibodies is rather large. The VH genes belong to the different variability subgroups; this also holds true for the VL repertoire although the single V kappa 4 gene may be used quite often. These variable segments often exhibit somatic mutations clustered in the CDR regions suggesting an antigen driven process. The monoclonal IgM is produced by a B cell clone which may be undetectable in the majority of patients. Nevertheless, it is possible to detect clonal B cells in the blood which have the remarkable capacity to differentiate spontaneously in vitro to plasma cells. This process is dependent upon an IL6-autocrine differentiation pathway which may be modulated by some drugs such as interferons or all trans retinoic acid.
Brouet
J C
JC
Service d'Immuno-Hématologie, Laboratoire d'Immunopathologie, Hôpital Saint-Louis, Paris.
D000911:Antibodies, Monoclonal / Q000737:chemistry / Q000145:classification / Q000502:physiology*; D001402:B-Lymphocytes / Q000473:pathology; D002454:Cell Differentiation; D006801:Humans; D007075:Immunoglobulin M / Q000737:chemistry / Q000145:classification / Q000502:physiology*; D007372:Interferons / Q000494:pharmacology; D010523:Peripheral Nervous System Diseases / Q000276:immunology / Q000503:physiopathology*; D010950:Plasma Cells / Q000473:pathology; D014212:Tretinoin / Q000494:pharmacology; D008258:Waldenstrom Macroglobulinemia / Q000276:immunology
D000911:Antibodies, Monoclonal; D007075:Immunoglobulin M; D014212:Tretinoin; D007372:Interferons