title: Malnutrition, pharmaconutrition and other considerations in AL amyloidosis, a rare disease with masquerading symptoms and usually delayed diagnosis.
abstract: 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.
authors.affiliation: Department of Radiology, Hospital Universitario Sant Joan, Alicante.. angelesfrnc@yahoo.com.
authors.forename: Ángeles
authors.initials: Á
authors.lastname: Franco-López
authors.affiliation: Member, Royal Academy of Medicine and Surgery of Valladolid. IBIOMED, Institute of Biomedicine, University of Leon, Spain.. jesus@culebras.eu.
authors.forename: Jesús M
authors.initials: JM
authors.lastname: Culebras
mesh_terms: D000686:Amyloidosis / Q000150:complications* / Q000175:diagnosis* / Q000628:therapy; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D057210:Delayed Diagnosis; D055951:Functional Food; D006331:Heart Diseases / Q000209:etiology; D006801:Humans; D008297:Male; D044342:Malnutrition / Q000209:etiology*; D008875:Middle Aged; D018529:Nutritional Support; D035583:Rare Diseases; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents