@entity1582 was diagnosed in 2 @entity1 , @entity1 aged 61 and 43 respectively. The first @entity1 , who had a @entity1985 , died soon after diagnosis as the disease appeared to be already widespread. The second @entity1 was still alive at the last follow-up, 17 years after diagnosis, because of effective elimination of her light chains by high-dose chemotherapy. @entity1582 is a rare, but severe, @entity262 with high mortality. Its aetiology lies in deregulated plasma cells producing excessive numbers of free immunoglobulin light chains. These light chains are the precursor proteins of amyloid fibrils. Amyloid fibrils are deposited extracellularly in tissue leading to organ dysfunction. Symptomatology is diverse, often non-specific, and generally not very well-known. Therefore, the diagnosis is often delayed for a long time. This is unfortunate, as high-dose chemotherapy targeted at elimination of the precursor protein considerably improves prognosis. However, this type of therapy can only be given in @entity1 with limited and moderately progressive disease.

[ XXXX and its treatment by eliminating the precursor protein].

@entity1582 :: ('MESH:D009101', 'Disease') :: ['AL amyloidosis'] @entity1 :: ('9606', 'Species') :: ['women', 'patients', 'patient'] @entity1985 :: ('MESH:D009404', 'Disease') :: ['nephrotic syndrome'] @entity262 :: ('MESH:D034721', 'Disease') :: ['systemic disease']
@entity1582 :: (MESH:D009101,Disease) :: ['AL-amyloidosis']
