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AL amyloidosis, also called light-chain amyloidosis, is a rare plasma cell disorder in which abnormal immunoglobulin light chains produced by a plasma cell clone misfold and aggregate into insoluble amyloid fibrils that deposit in organs and tissues. The condition presents in two forms: primary systemic amyloidosis, which involves multiple organs, and primary localized amyloidosis, which is restricted to a single organ. It affects approximately 1 to 9 individuals per 100,000.
Symptoms in AL amyloidosis reflect organ involvement by amyloid fibril deposition. The primary systemic form affects multiple organ systems. Specific phenotype data are not provided in this packet; clinical manifestations and their severity vary substantially among affected individuals based on which organs are involved.
Data assembled from 5 of 12 sources · Last updated Sep 20, 2026, 3:00 PM UTC
European rare disease database
Genetic and Rare Diseases Info Center
AL amyloidosis arises as an acquired condition in which a plasma cell clone produces abnormal monoclonal immunoglobulin light chains that aggregate into amyloid fibrils. The condition is not caused by an inherited genetic change. No causative genes are listed in this packet.
Evaluation typically involves tissue biopsy to confirm amyloid deposition and characterize the amyloid type as light-chain (AL) origin. Assessment of affected organ systems is part of staging. Pathologic confirmation is central to establishing the diagnosis.
Treatment planning for AL amyloidosis depends on organ involvement, disease severity, and individual health factors. Management typically involves a multidisciplinary team and may include systemic treatment approaches targeting the plasma cell clone and organ-supportive care. Treatment goals are individualized and may focus on disease control and preservation of organ function.
67 trials found
Outcomes depend on the extent and severity of organ involvement, particularly involvement of critical organ systems, and on response to treatment. Survival outcomes vary considerably among affected individuals. Access to specialized oncology and organ-specific care may influence outcomes.
AL amyloidosis is an active area of clinical investigation. Numerous certified active trial records are present, spanning multiple phases and examining drug therapy, biologic therapy, and investigational approaches. Information on current trials is available through ClinicalTrials.gov.
AI-curated news mentioning AL amyloidosis
Updated Sep 9, 2026
A Loveland man credits an investigational CAR-T cell therapy with improving his condition related to AL amyloidosis, a rare disease characterized by abnormal protein buildup. He participated in a clinical trial at The James Cancer Hospital, although the treatment is not yet widely available.
A recent study highlights raccoon eye as a unique presenting sign of AL amyloidosis linked to a low-burden clonal plasma cell neoplasm. This finding may enhance diagnostic awareness for clinicians encountering similar symptoms.
A recent study highlights the occurrence of cardiac tamponade due to lymphocytic constrictive pericarditis in patients with primary AL amyloidosis, even in the absence of myocardial involvement. This finding may influence future diagnostic and treatment approaches for this rare condition.
A recent pooled analysis evaluates the efficacy and safety of BCMA-targeted therapies in patients with relapsed or refractory AL amyloidosis. This study provides insights into treatment options for a challenging condition, although further research is needed to confirm findings.
Promising results from CAR T-cell therapy targeting BCMA in light chain amyloidosis show excellent response rates and safety profiles. However, regulators are requiring randomized clinical trials to validate these findings, posing challenges for this rare disease.