The American Society of Hematology (ASH) has published new clinical practice guidelines aimed at improving the early, timely, and accurate diagnosis of light chain (AL) amyloidosis.1
The guidelines were published January 28, 2026, in Blood Advances and outline best practices to support clinicians in recognizing and diagnosing AL amyloidosis, a disease frequently missed or diagnosed late due to its heterogeneous presentation.1
“These guidelines will be a valuable resource not only for hematologists, but for clinicians across other specialties who care for patients with AL amyloidosis,” said Robert Negrin, MD, ASH President, in a statement. “Because this is a rare and often underrecognized disorder, these recommendations are particularly important for coordinating care and increasing awareness.”
AL amyloidosis is typically a multisystem disease, with amyloid deposits affecting multiple organs. The heart and kidneys are most commonly involved, potentially resulting in cardiomyopathy, renal failure, and pleural or pericardial effusions. Multiorgan dysfunction is strongly associated with poorer outcomes and reduced survival, while 51% to 80% of patients present with cardiac involvement, a major cause of morbidity and mortality in these patients.2,3
The pathogenesis of AL amyloidosis is caused by the misfolding and tissue deposition of monoclonal immunoglobulin light chains produced by abnormal plasma cells. These amyloid deposits, along with circulating free light chains, disrupt normal tissue structure and function, leading to progressive organ damage and increased risk of death, particularly when diagnosis is delayed.2,3,4
In the past, patients have reported diagnostic delays of >1 year from symptom onset, especially since the presenting symptoms are non-specific, and first-line clinicians may not have disease awareness.4
For example, one primary risk factor for AL amyloidosis is increased age, with a median age at diagnosis of approximately 64 years. Since the disease often presents later in life, nonspecific symptoms such as fatigue, weight loss, or reduced appetite are frequently misattributed to aging or more common chronic conditions, contributing to prolonged diagnostic delays.2