Light chain deposition disease
Light chain deposition disease (LCDD) is a rare disease.
It can affect multiple organ systems.
- renal manifestations of light chain deposition disease - renal involvement is a constant feature, resulting in proteinuria with or without nephrotic syndrome and renal failure.
- hepatic manifestations of light chain deposition disease
- cardiac manifestations of light chain deposition disease
- pulmonary manifestations of light chain deposition disease
Epidemiomology
It mostly affects middle age patients and there is no recognized gender predilection
Pathology
It is characterized by the deposition of a nonfibrillary, amorphous material that does not have β-pleated sheet configuration and consequently does not bind Congo red as amyloidosis. This eosinophilic material corresponds to monotypic immunoglobulin light chains deposits. The term “LCDD” is restricted to the nonamyloid forms of light chain deposition. Light chains are secreted by a clone of plasma cells
Associations
About 75% of LCDD occur in association with multiple myeloma or a lymphoproliferative disease, especially Waldenström macroglobulinemia.
Histology and etymology
The condition was recognized in 1976 by Randall.
