Primary Amyloidosis With Renal Involvement: Outcomes in 77 Consecutive Patients at a Single Center

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Abstract

Background

Outcomes in primary amyloid renal patients are of interest as the era of monoclonal antibody therapies begins.

Patients and Methods

We studied 77 consecutive primary amyloid renal patients (58% men) for renal progression (end stage renal disease [ESRD]), renal response (RR), and overall survival (OS).

Results

At diagnosis median age was 63 (range, 35-81) years, estimated glomerular filtration rate 70 mL/min (range, 5-114), difference between involved and uninvolved free light chains 127 mg/L (range, 1-9957), ESRD 4%, renal stage 2 and 3 78%, and cardiac stage 2 and 3 56%. Ninety-six percent received bortezomib and 44% stem cell transplantation as well as bortezomib, 68% achieved complete or very good partial hematologic response (CR/VGPR), 34% had ESRD, and 39% RR. Median times to ESRD and RR were 18 (range, 3-81) and 12 (range, 2-30) months, respectively. Median OS was not reached in this cohort and was not reached from onset of ESRD. More than two-thirds of patients with ESRD also achieved CR/VGPR. In those without ESRD at diagnosis, baseline creatinine and absent RR predicted progression to ESRD in multivariate Cox regression analysis, whereas CR/VGPR predicted RR. In multivariate Cox regression analysis, cardiac stage and achievement of CR/VGPR predicted OS, enabling construction of a prognostic model.

Conclusion

Anti-plasma cell therapies provide a definite albeit limited benefit and new approaches to amyloid-related organ dysfunction are needed.

Micro-Abstract

In primary amyloidosis toxic clonal immunoglobulins damage and deposit in the heart, kidneys and other organs. We report on 77 consecutive patients with primary amyloidosis and renal involvement treated and followed over a 7-year period. Although two thirds achieved deep hematologic responses with chemotherapy, over a third progressed to ESRD, an outcome highlighting the hitherto unmet need for anti-amyloid therapy.

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