Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL): A consensus opinion from the 10th International Symposium on Amyloid and Amyloidosis

Best use of cardiac biomarkers in patients with AL amyloidosis and renal failure

“…All the patients underwent a complete physical examination and echocardiography. Amyloid cardiac involvement was defined according to the International Society of Amyloidosis guidelines as mean left ventricular wall thickness >12 mm in the absence of other cardiac causes [18]. The presence of symptoms of heart failure was defined as a New York Heart Association Class 2 or greater.…”

Treatment of patients with advanced cardiac AL amyloidosis with oral melphalan, dexamethasone, and thalidomide

“…Heart involvement was defined as mean left ventricular wall thickness >12 mm at echocardiography in the absence of other cardiac diseases, according to the International Society for Amyloidosis criteria [10]. All the patients underwent physical examination, high-resolution serum, and urine immunofixation electrophoresis, circulating free light-chain (FLC) measurement, complete blood count, assessment of renal and liver function, echocardiography, and 24-h electrocardiogram (Holter ECG).…”

“…Response was defined according to the International Society for Amyloidosis criteria [10]. Hematologic response was defined as a >50% reduction of the concentration of the amyloidogenic FLC and complete remission (CR) as the disappearance of the monoclonal component at serum and urine immunofixation, normal FLC ratio, and bone marrow plasma cell of <5%.…”

Consensus guidelines for the conduct and reporting of clinical trials in systemic light-chain amyloidosis

“…32,33 Because patients post-SCT frequently achieved profound complete hematologic responses with improved organ function, the objective scoring of hematologic and organ responses was deemed important in the 2005 consensus opinion of AL investigators. 34 Questions of validation were not addressed at that time and the criteria referred specifically to newly diagnosed patients.…”

“…Creatinine and creatinine clearance must not worsen by 25% over baseline 50% increase (at least 1 g/day) of 24-h urine protein to 41 g/ day or 25% worsening of serum creatinine or creatinine clearance Liver 50% decrease in abnormal alkaline phosphatase value Decrease in liver size radiographically at least 2 cm 50% increase of alkaline phosphatase above the lowest value Organ responses Organ disease that is considered to be quantifiable for response includes cardiac disease, renal disease with proteinuria, and hepatic disease (Table 2). 34,35 Amyloid cardiomyopathy leads to a concentric increase in ventricular wall thickness; however, change in mean wall thickness measured by echocardiogram has not yet been shown to be a useful clinical end point. Worsening left ventricular ejection fraction, while a harbinger of poor outcome, is not useful either.…”