Resumo

FRANCO-LOPEZ, Ángeles  e  CULEBRAS, Jesús M.. Malnutrition, pharmaconutrition and other considerations in AL amiloidosis, a rare disease with masquerading symptoms and usually delayed diagnosis. Nutr. Hosp. [online]. 2015, vol.31, n.6, pp.2337-2345. ISSN 1699-5198.  https://dx.doi.org/10.3305/nh.2015.31.6.7851.

Amyloid Light-chain (AL) amyloidosis is a very rare disease. Nutritional and pharmaconutrional aspects are described. Nutrition repletion of malnourished AL patients is an essential strategy for improving treatment efficacy and clinical outcomes. Early diagnosis of AL amiloidosis 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.

Palavras-chave : AL amyloidosis; Cardiac amyloidosis.

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