A renal staging system for the prediction of ESRD using universally available measurements (i.e., proteinuria and eGFR) was validated in patients with AL amyloidosis and being used in clinical practice. Approximately 57–80% of AL amyloidosis patients have renal involvement at presentation, and up to ~40% of these patients require renal replacement therapy during the course of their disease. The accumulation of amyloid fibrils disrupting tissue structure and direct toxicity from circulating precursors eventually leads to organ failure and death. It is a systemic disease that affects various organs and is associated with an underlying clonal B-cell disorder (most commonly plasmacytic).
Light chain (AL) amyloidosis is a result of extracellular deposition of pathologic, insoluble fibrils that are formed from abnormal light chains (LCs) or LC fragments. Patients with hematologic CR or VGPR have the most favorable outcomes, and these patients should be considered for renal transplantation. Renal transplantation in selected AL amyloidosis patients is associated with extended overall and renal graft survival. Although 69 patients (29%) experienced hematologic relapse, treatment effectively prevented graft loss in the majority (87%). VGPR there was no difference in overall or graft survival. The frequency and time to amyloid recurrence in the graft was also lower (16% vs 37%, p = 0.01) and longer (median time not achieved vs 10 years, p = 0.001) in the CR + VGPR group. Median graft survival was 7.8 years and was better in the CR + VGPR group (8.3 vs 5.7 years, HR: 1.4, P = 0.05 ).
With a median follow-up of 8.5 years, the median overall survival from renal transplantation was 8.6 years and was significantly longer in patients with complete and very good partial hematologic responses (CR + VGPR) compared to less than VGPR (9 versus 6.8 years HR: 1.5, P = 0.04 ) at renal transplantation. This multicenter, observational study from five countries includes 237 patients with AL amyloidosis who underwent renal transplantation between 19.
There is a critical need to identify patients in this population who benefit from renal transplantation.
Outcomes of renal transplantation in patients with AL amyloidosis: an international collaboration through The International Kidney and Monoclonal Gammopathy Research Group
0 kommentar(er)
