Validation of new renal staging system in AL amyloidosis treated with high dose melphalan and stem cell transplantation

Predictive value of the new renal response criteria in AL amyloidosis treated with high dose melphalan and stem cell transplantation

“…Median overall survival was 7.9 years (95% CI 6.9–9.1) following HDM/SCT, which is favorable to 3.9 years (Pavia cohort) and 2.5 years (Heidelberg cohort) as reported by Palladini et al [1]. Similarly, renal survival was also longer in our cohort compared to that reported in Palladini et al [1, 4]. Median renal survival was 13.5 years with a median follow-up of 6.9 years (range, 0.4–20.8).…”

“…Median renal survival was 13.5 years with a median follow-up of 6.9 years (range, 0.4–20.8). The 2-year dialysis risk was also lower compared to the Pavia – Heidelberg cohort: 0% in renal stage I, 3% in stage II and 14% in stage III renal disease [4, 5]. Median time to renal response, defined as 30% decrease in proteinuria or drop of proteinuria below 0.5 g/24 h in the absence of ≥25% decrease in eGFR, was 12.3 months (range, 2.9–146.7), while median time to renal progression was 13.3 months (range, 0.1–149.7).…”

Long-term outcome of kidney transplantation in AL amyloidosis

“…1,2 The accumulation of amyloid fibrils disrupts tissue structure and can eventually lead to organ failure and death. More than two-thirds of patients have renal involvement at presentation, [3][4][5] and 19% to 42% of these patients will require renal replacement therapy (RRT) during the course of their disease. 3,6 Until the mid-1990s the treatment of AL amyloidosis was limited to melphalan and prednisone.…”

“…More than two-thirds of patients have renal involvement at presentation, [3][4][5] and 19% to 42% of these patients will require renal replacement therapy (RRT) during the course of their disease. 3,6 Until the mid-1990s the treatment of AL amyloidosis was limited to melphalan and prednisone. Hematologic response to this therapy was insignificant and delayed, resulting in a median overall survival (OS) of only 12.2 to 18 months from the time of diagnosis.…”

High-Dose Melphalan and Stem Cell Transplantation in Patients on Dialysis Due to Immunoglobulin Light-Chain Amyloidosis and Monoclonal Immunoglobulin Deposition Disease

“…Among the 36 human fibril proteins associated with human disease established by the Nomenclature Committee of the International Society of Amyloidosis in 2016, the most frequent is immunoglobulin light chain (AL) systemic amyloidosis [2]. Although many different organs can be involved in systemic AL amyloidosis, renal involvement is the most common clinical manifestation, affecting roughly three fourths of patients [3], and 15% to 31% become dialysis-dependent, with a median survival of 8 of 39 months from the initiation of dialysis [4,5].…”