Presentation and Outcomes of Localized Immunoglobulin Light Chain Amyloidosis: The Mayo Clinic Experience

doi:10.1016/j.mayocp.2017.02.016

Mayo Clinic Proceedings

Volume 92, Issue 6, June 2017, Pages 908-917

https://doi.org/10.1016/j.mayocp.2017.02.016 Get rights and content

Abstract

Objective

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

Patients and Methods

We included all patients with AL_L seen at Mayo Clinic in Rochester, Minnesota, from January 1, 1968, through June 30, 2014. The diagnosis of AL_L 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 AL_L. 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.

Section snippets

Patients and Inclusion Criteria

From January 1, 1968, through June 30, 2014, clinical laboratory and treatment data were extracted from a prospectively maintained database. Patients before 1968 were excluded because immunofixation and immunoelectrophoresis were not available before that time. The Mayo Foundation Institutional Review Board approved the study, and all the patients consented to have their medical records reviewed according to institutional review board practices. The diagnosis of AL_L was predicated on the

Patient Characteristics and Presentation of AL_L

Of 5551 patients with AL seen at Mayo Clinic in Rochester, Minnesota, during the study period, 413 (7%) had AL_L. The baseline characteristics of patients according to involved sites are shown in Table 1. Fifty percent of patients were diagnosed before December 1, 2000. The most common site involved was urothelial tissue (bladder, ureter, renal pelvis) (Table 1). Of 413 patients, 243 (59%) did not have typing. In addition to negative immunofixation of the serum and urine, 264 (64%) of patients

Discussion

In this large series of patients with AL_L, for the first time, we systematically described long-term outcomes with first-line therapy and recurrence patterns for these patients. We note that urothelial and laryngeal AL_L are more likely to have repeated reoccurrences after first-line therapy. Finally, we demonstrate that if patients present with “typical” sites of AL_L (tracheobronchial, pharyngeal, laryngeal, and urothelial), their initial laboratory evaluation to exclude systemic AL could be

Conclusion

In this large series of AL_L, we found that AL_L is rare but can cause substantial morbidity and, in rare cases, mortality. Excision and radiotherapy are associated with high response rates for symptomatic patients, but a more conservative approach is reasonable for asymptomatic patients. If simple hematologic test results are negative, more comprehensive testing is not necessary and systemic progressions do not occur. Lifelong follow-up, however, is important because locoregional recurrences are

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Original articlePresentation and Outcomes of Localized Immunoglobulin Light Chain Amyloidosis: The Mayo Clinic Experience

Abstract

Objective

Patients and Methods

Results

Conclusion

Section snippets

Patients and Inclusion Criteria

Patient Characteristics and Presentation of ALL

Discussion

Conclusion

Lancet Haematol

J Lab Clin Med

Hum Pathol

BBA Clin

Int J Radiat Oncol Biol Phys

Localized AL amyloidosis: a suicidal neoplasm?

Ups J Med Sci

Carpal tunnel syndrome: a common initial symptom of systemic wild-type ATTR (ATTRwt) amyloidosis

Amyloid

Immunoglobulin light chain amyloidosis is diagnosed late in patients with preexisting plasma cell dyscrasias

Am J Hematol

Laryngeal amyloidosis as a cause of death

Amyloid

Prevalence of monoclonal gammopathy of undetermined significance

N Engl J Med

Prior autoimmune disease and risk of monoclonal gammopathy of undetermined significance and multiple myeloma: a systematic review

Cancer Epidemiol Biomarkers Prev

Original article
Presentation and Outcomes of Localized Immunoglobulin Light Chain Amyloidosis: The Mayo Clinic Experience

Patient Characteristics and Presentation of AL_L