SYSTEMIC LAMBDA-LIGHT CHAIN DEPOSITION DI SEASE (LCDD) WITH HYPERTROPHIC CARDIOMYOPATHY WITHOUT RENAL-FAILURE

LCDD has been identified as a systemic entity characterized by general ized, extracellular, Congo red negative light chain (LC) deposits, whi ch differ from amyloid substance. Above all kidney (renal failure/neph rotic syndrome), less frequently liver, heart and skin are involved. M onoclonal LC-disease (usually kappa-type) and myeloma make the basis o f LCDD. It is pointed out, that exceptionally LCDD-cardiomyopathy-with out nephropathy - leads to irresponsive global cardiac failure. The ev idence of monoclonal lambda-chains in serum and urine was as striking as diffuse plasmacytosis without myeloma. For therapeutic reasons susp icion of cardiac or renal LCDD should be ruled out by biopsy and immun o-electrophoresis.