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AL amyloidosis is caused by the deposition of abnormal antibody free light chains. The abnormal light chains are produced by monoclonal plasma cells, and, although AL amyloidosis can occur without diagnosis of another disorder, it is often associated with other plasma cell disorders, such as multiple myeloma and Waldenström's macroglobulinemia. [6]
Survival diminishes with increasing stage, but recent advancements in treatments have improved median survival rates for stages I, II, and III, to 91.2, 60, and 7 months respectively. [45] Outcomes in a person with AA amyloidosis depend on the underlying disease, organ(s) affected, and correlate with the concentration of serum amyloid A protein ...
Light chain (AL-CM) Prognosis: For light-chain amyloidosis early detection leads to best possibility of therapies prolonging the period of remission. [3] Well treated light chain cardiac amyloidosis has a 4-year survival rate of around 90%. [5] In patients that undergo stem cell transplant the average survival time increases to 10 years. [5]
Light chain multiple myeloma is diagnosed in patients who have: a) the criteria for diagnosis of multiple myeloma except having a serum free light chain ratio outside the normal range of 0.26 to 1.65 without evidence of an intact immunoglobulin or free heavy chain; or b) an extreme free light chain ratio, i.e. outside the range of 0.02 to 100 ...
5-year survival rate of 10% [2] Amyloid cardiomyopathy ( stiff heart syndrome ) [ 5 ] is a condition resulting in the death of part of the myocardium (heart muscle). It is associated with the systemic production and release of many amyloidogenic proteins , especially immunoglobulin light chain or transthyretin (TTR). [ 6 ]
The onset of FAC caused by aggregation of the V122I mutation and wild-type TTR, and senile systemic amyloidosis caused by the exclusive aggregation of wild-type TTR, typically occur after age 60. Greater than 40% of these patients present with carpal tunnel syndrome before developing ATTR-CM.
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