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

The Clone Wars: Diagnosing and Treating Dysproteinemic Kidney Disease in the Modern Era

“…Over the last decade renal staging and renal response criteria have been developed to predict the risk of end stage kidney disease in AL amyloidosis based on baseline kidney function and proteinuria (Stage I: estimated glomerular filtration rate (eGFR) ≥ 50 mL/min/1.73 m 2 and <5 g/24 h proteinuria; Stage II: either eGFR < 50 mL/ min/1.73 m 2 or ≥5 g/24 h proteinuria; Stage III: eGFR < 50 mL/min/1.73 m 2 and ≥5 g/24 h proteinuria) [65,70,75]. At 6 or 12 months post-treatment, the cutoff for predicting favorable kidney outcome with therapy was a~30% decrease in proteinuria (or a drop of proteinuria below 0.5 g/24 h) without worsening of eGFR, while a~25% decrease in eGFR correlated with worse renal outcome [73,75].…”

“…The most aggressive form of treatment is high-dose melphalan with autologous stem cell transplantation (HDM/SCT) but due to concerns about treatment-related morbidity and mortality, only 20% of newly diagnosed patients with AL amyloidosis are eligible for, and eventually undergo, this form of therapy [70,83]. A rapid decrease in the circulating LC concentration in patients who achieve a hematologic CR or VGPR can reverse proteinuria and renal dysfunction over time with median time from hematologic response to renal response of~10-11 months [73,75,84]. Unfortunately, some patients do not achieve renal organ response despite experiencing hematologic response.…”