Presentation and Outcomes of Localized Immunoglobulin Light Chain Amyloidosis

摘要: Abstract Objective To describe treatment types, outcomes, and relapse patterns in patients with localized immunoglobulin light chain amyloidosis (AL L ). Patients Methods We included all AL seen at Mayo Clinic Rochester, Minnesota, from January 1, 1968, through June 30, 2014. The diagnosis of was predicated on the presence a Congo red–positive biopsy specimen negative serum urine immunofixation. Treatment response categories were response, stability, progression. Localized systemic progressions defined as progression disease site origin or appearance clonal plasma cells bone marrow sample, respectively. Results Of 5551 AL, 413 (7%) had . most common involved urothelial tissue (n=85, 21%), followed by larynx (n=57, 14%). Coexisting autoimmune diseases reported 7% (n=28). first-line excision amyloid deposits (61%), observation supportive care (28%). When considering symptomatic only (n=284), 205 (72%) improved, 23 (8%) stable disease, 55 (19%) could not be evaluated for response. Ten-year survival 78% different that general population. There no progressions, but 17% (n=72) Conclusion is associated relatively distinct pattern organ involvement. initial laboratory evaluation to exclude limited immunofixation patients. Recurrence after therapy common, long-term outcomes are excellent.