[Light chain diseases--pathogenetic forms, diagnostic criteria. A suggestion for classification].
The clinical significance of the BJ-protein is quite variable: from an asymptomatic course of the light chain proteinuria for a long period of time to specific tissue deposits with serious organ damages and dysfunction. On the basis of literature data a classification of the light chain diseases is proposed: 1. Essential (bening) BJ-proteinuria; 2. Smoldering BJ-myeloma; 3. Light chain disease--BJ myeloma; 4. Light (and heavy) chain deposition disease: LC granular thick deposits mostly k type on electron microscopy, belonging to the constant region of the polypeptide chain and actively reacting with antiserums, not possessing tincturial properties; 5. LC cast nephropathy--LC coprecipitates in the tubular system; 6. LC crystaline deposition disease--specific crystaloid structures (in the Fanconi syndrome and experimental nephropathies); 7. AL-amyloidosis: LC precursor mostly lambda type from the variable region with specific fibrilar structure and typical tincturial properties. The suggested classification would resolve the diagnostic difficulties and determine the correct therapeutic management.
Goranov
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D000686:Amyloidosis / Q000145:classification / Q000175:diagnosis* / Q000276:immunology; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000276:immunology*; D008854:Microscopy, Electron; D011507:Proteinuria / Q000145:classification / Q000175:diagnosis* / Q000276:immunology
D007147:Immunoglobulin Light Chains