Light chain deposition disease
| Light chain deposition disease | |
|---|---|
| Classification and external resources | |
| Specialty | oncology |
| ICD-10 | ICD-10-CM |
| ICD-9-CM | 203.8 |
| eMedicine | 202585/ |
Light chain deposition disease (LCDD) is a rare blood cell disease which is characterized by deposition of fragments of infection-fighting immunoglobulins, called light chains (LCs), in the body. LCs are normally cleared by the kidneys, but in LCDD, these light chain deposits damage organs and cause disease. The kidneys are almost always affected and this often leads to kidney failure. About half of people with light chain deposition disease also have multiple myeloma.[1][2] Unlike in AL amyloidosis, in which light chains are laid down in characteristic amyloid deposits, in LCDD, light chains are deposited in non-amyloid granules.[3]
Signs and symptoms
The kidney is the organ most frequently affected. Proteinuria, loss of protein in the urine, is characteristic. More than 90% of people with LCDD develop kidney failure, often with rapid progression of disease.[3]
Light chains may be deposited in many other organs and may or may not result in any symptoms. Other than the kidneys, liver and heart are the most commonly involved organs. Deposition of light chains in the liver may lead to hepatomegaly, an enlarged liver, or rarely portal hypertension or liver failure. The heart is affected in up to 80% of patients with LCDD, and may cause arrythmias and congestive heart failure.[3]
Prognosis
The median time to progression to end stage renal disease is 2.7 years. After 5 years, about 37% of patients with LCDD are alive and do not have end stage renal disease.[1]
Treatment
There is no standard treatment for LCDD. High-dose melphalan in conjunction with autologous stem cell transplantation has been used in some patients. A regimen of bortezomib and dexamethasone has also been examined.[1]
References
- 1 2 3 Kastritis (February 2009). "Treatment of light chain deposition disease with bortezomib and dexamethasone". Haematologica 94 (2): 300–302. doi:10.3324/haematol.13548. PMC 2635400. PMID 19066331.
- ↑ UNC Kidney Center. "Light Chain Deposition Disease". UNC. Retrieved 29 November 2011.
- 1 2 3 Ronco (July 2001). "Light Chain Deposition Disease: A Model of Glomerulosclerosis Defined at the Molecular Level". J Am Soc Nephrol 12 (7): 1558–1565. PMID 11423587.
External links
- National Institutes of Health Genetic and Rare Diseases Information Center
- Emedicine
- Useful information on LCDD