AL amyloidosis

AL amyloidosis, or primary amyloidosis, is the most common form of systemic amyloidosis in the US.

It occurs in 5 to 15% of people with multiple myeloma.

The "AL" refers to amyloid light chain.

Presentation
The Bence Jones proteins produced are necessary to produce amyloidosis but other factors are required for amyloidosis to occur. Most people with AL amyloidosis do not have multiple myeloma, or any other B cell tumour. They often have a serum gammopathy, and it is unknown whether these cases will ultimately evolve into myeloma, as these people often die from their amyloidosis before malignant transformation occurs.

Treatment
Treatment can involve application of chemotherapy similar to that used in multiple myeloma.

A combination of bortezomib and dexamethasone has been proposed, as has melphalan and dexamethasone.