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Start free with EleplanAL amyloidosis
ORPHA:85443Disease
Also called Light-chain amyloidosis
What it is
A clonal B-cell disorder characterized by the aggregation and deposition of insoluble amyloid fibrils derived from misfolding of monoclonal immunoglobulin light chains. It usually presents as systemic AL amyloidosis with involvement of one or more parenchymal organ(s) and, less frequently, as localized amyloidosis with usually nodular deposits restricted to a single organ and/or system.
Key facts
- Prevalence
- 1-9 / 100 000
- Age of onset
- Adult
- Inheritance
- Not applicable
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Common30–79%
24- Abnormal atrioventricular conduction
- Abnormal EKG
- Abnormality of taste sensation
- Abnormal pulmonary interstitial morphology
- Albuminuria
- Bundle branch block
- Decreased QRS voltage
- Erectile dysfunction
- Hepatic amyloidosis
- Hypertrophic cardiomyopathy
- Increased circulating immunoglobulin concentration
- Increased circulating NT-proBNP concentration
- Increased circulating troponin I concentration
- Increased circulating troponin T concentration
- Left ventricular hypertrophy
- Malabsorption
- Monoclonal light chain cardiac amyloidosis
- Nephrotic range proteinuria
- Nephrotic syndrome
- Periorbital purpura
- Proteinuria
- Renal interstitial amyloid deposits
- Sensorimotor neuropathy
- Weight loss
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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