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Presentation and Outcomes of Localized Immunoglobulin Light Chain Amyloidosis

The Mayo Clinic Experience

      Abstract

      Objective

      To describe treatment types, outcomes, and relapse patterns in patients with localized immunoglobulin light chain amyloidosis (ALL).

      Patients and Methods

      We included all patients with ALL seen at Mayo Clinic in Rochester, Minnesota, from January 1, 1968, through June 30, 2014. The diagnosis of ALL was predicated on the presence of a Congo red–positive biopsy specimen and negative serum and urine immunofixation. Treatment response categories were response, stability, and progression. Localized and systemic progressions were defined as progression of disease at the site of origin or appearance of clonal plasma cells in a bone marrow biopsy sample, respectively.

      Results

      Of 5551 patients with AL, 413 (7%) had ALL. The most common site involved was urothelial tissue (n=85, 21%), followed by the larynx (n=57, 14%). Coexisting autoimmune diseases were reported in 7% of patients (n=28). The most common first-line treatment was excision of the amyloid deposits (61%), followed by observation or supportive care (28%). When considering symptomatic patients only (n=284), 205 (72%) improved, 23 (8%) had stable disease, and 55 (19%) could not be evaluated for response. Ten-year survival was 78% and was not different from that of the general population. There were no systemic progressions, but 17% of patients (n=72) had localized progression.

      Conclusion

      Localized AL is associated with a relatively distinct pattern of organ involvement. The initial laboratory evaluation to exclude systemic disease could be limited to serum and urine immunofixation in most patients. Recurrence after first-line therapy is common, but long-term outcomes are excellent.

      Abbreviations and Acronyms:

      AH/AL (heavy/light chain amyloidosis), AL (immunoglobulin light chain amyloidosis), ALL (localized immunoglobulin light chain amyloidosis), BSC (best supportive care), DMSO (dimethyl sulfoxide), IFE (immunofixation), MALT (mucosa-associated lymphoid tissue), MGUS (monoclonal gammopathy of undetermined significance), NE (not evaluated), NOS (not otherwise specified), OS (overall survival), PFS (progression free survival), TTP (time to progression)
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