Pathogenetic implications

Bence Jones proteins play a direct role in myeloma-associated kidney disease and their measurement is of important prognostic significance. They are also involved in visceral diseases featuring tissue deposition of LC-related material. Combined study of serum and urine by sensitive methods reveals evidence of BJP in almost all cases of AL (amyloid light chain) amyloidosis. In LC deposition disease, tissue deposits of monoclonal LC (and of monoclonal heavy chains in some patients) correlate with the presence of a monoclonal population of bone marrow plasma cells, whatever the clinical context. In some cases, the LC are normal-sized and present in urine but in about 60-70% of patients they display an abnormal (short or enlarged by glycosylation) size, polymerize and are undetectable in serum and urine. In both instances there are numerous mutations in the variable (V) regions of the LC, especially in the hypervariable regions (with probably a direct pathogenic role), together with an abnormal subgroup distribution (VkIV predominance). V region abnormalities, with a V domain (of the VkI subgroup) highly resistant to proteolysis and prone to readily form crystals, are also involved in myeloma-associated Fanconi syndrome.

See also: Amyloid; Heavy chain diseases; Immunoelectrophoresis; Immunoglobulin structure; Lymphoma; Western blotting.

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