AL Amyloidosis: A dismal outcome related to usually delayed diagnosis
DOI:
https://doi.org/10.19230/jonnpr.2016.1.3.1012Keywords:
AL amiloidosis, Cardiac amyloidosisAbstract
Early diagnosis of AL amyloidosis is difficult to establish due to the fact that signs and symptoms appearing mimic other processes that delay the final correct histological diagnosis. Untreated patients with this disease have a dismal outcome, with a median survival of 10-14 months from diagnosis. The sooner the treatment is established the better the results are. Modern chemotherapeutical agents, based primarily in cyclophosphamide, bortethomid and dexametasone, produce a rapid, deep, and durable response in the majority of patients. Autologous stem cell transplantation remains restricted to selected patients who are generally without advanced cardiac amyloidosis. Protoclos intended to detect the disease at earlier stages should be established. In the case of carpal tunnel syndrome, red Congo stain in the removed tissues should be mandatory.
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