Immunoglobulin Light Chain Amyloidosis: 2026 Update on Diagnosis, Prognosis, and Treatment - PubMed
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- AL amyloidosis is a clonal plasma cell disorder with immunoglobulin light or heavy chain fragments deposited in tissues, affecting organs like the heart, kidneys, liver, and nerves.
- Diagnosis requires Congo red-stained tissue biopsy showing apple-green birefringence; organ biopsy is unnecessary in 85% of cases, but light chain verification is mandatory.
- Prognosis is staged using NT-proBNP/BNP, serum troponin, and free light chain differences, with 5-year survival rates ranging from 82% to 20% across four stages.
- First-line therapy includes daratumumab, bortezomib, cyclophosphamide, and dexamethasone, aiming for ≥ VGPR; consolidation options for non-responders include pomalidomide, stem cell transplant, and venetoclax.
- T-cell redirecting therapies (bispecific antibodies and CAR-T) show promise as future second-line treatments.
- Delayed diagnosis remains a challenge; an antibody targeting κ fibrils has shown benefits in cardiomyopathy patients.