light chain amyloidosis
E852351
immunoglobulin light chain deposition disease
plasma cell dyscrasia
protein misfolding disease
rare disease
systemic amyloidosis
Light chain amyloidosis is a rare systemic disorder in which misfolded immunoglobulin light chains form amyloid deposits in organs such as the heart and kidneys, leading to progressive organ dysfunction.
All labels observed (1)
| Label | Occurrences |
|---|---|
| light chain amyloidosis canonical | 1 |
How this entity was disambiguated
This entity first appeared as the object of triple T10292098 — resolving that mention is where its identity was fixed. The disambiguator weighed these candidate entities and picked the highlighted one (or “None”, minting a new entity). This is how homonymy is resolved: the same surface form can point to different entities.
NED1
Entity disambiguation (via context triple)
gpt-5-mini-2025-08-07
Target entity: light chain amyloidosis Context triple: [CD38, therapeuticArea, light chain amyloidosis]
-
A.
Leigh-Mallory
Leigh-Mallory is the surname of a prominent British military family, most notably borne by Air Chief Marshal Trafford Leigh-Mallory, a senior Royal Air Force commander during the Second World War.
-
B.
LDH
LDH is the IATA airport code for Lord Howe Island Airport, which serves Lord Howe Island in New South Wales, Australia.
-
C.
Hipple
Hipple is the birth surname of American film and television actor Hugh Marlowe.
-
D.
Meckingsen
Meckingsen is a village-level district that forms part of the town of Soest in North Rhine-Westphalia, Germany.
-
E.
CLL
CLL is the IATA airport code for Easterwood Airport, a regional airport serving College Station, Texas.
- F. None of above. chosen
- G. Unsure - the case is ambiguous/there is not enough information to decide.
NED2
Entity disambiguation (via description)
gpt-5-mini-2025-08-07
Target entity: light chain amyloidosis Target entity description: Light chain amyloidosis is a rare systemic disorder in which misfolded immunoglobulin light chains form amyloid deposits in organs such as the heart and kidneys, leading to progressive organ dysfunction.
-
A.
Leigh-Mallory
Leigh-Mallory is the surname of a prominent British military family, most notably borne by Air Chief Marshal Trafford Leigh-Mallory, a senior Royal Air Force commander during the Second World War.
-
B.
LDH
LDH is the IATA airport code for Lord Howe Island Airport, which serves Lord Howe Island in New South Wales, Australia.
-
C.
Hipple
Hipple is the birth surname of American film and television actor Hugh Marlowe.
-
D.
Meckingsen
Meckingsen is a village-level district that forms part of the town of Soest in North Rhine-Westphalia, Germany.
-
E.
CLL
CLL is the IATA airport code for Easterwood Airport, a regional airport serving College Station, Texas.
- F. None of above. chosen
Statements (64)
| Predicate | Object |
|---|---|
| instanceOf |
immunoglobulin light chain deposition disease
ⓘ
plasma cell dyscrasia ⓘ protein misfolding disease ⓘ rare disease ⓘ systemic amyloidosis ⓘ |
| affectsOrgan |
autonomic nervous system
ⓘ
bone marrow ⓘ gastrointestinal tract ⓘ heart ⓘ kidneys ⓘ liver ⓘ peripheral nerves ⓘ soft tissues ⓘ |
| hasAbbreviation | AL amyloidosis ⓘ |
| hasAssociation |
monoclonal gammopathy of undetermined significance
ⓘ
multiple myeloma ⓘ |
| hasBiomarker |
abnormal serum free light chain ratio
ⓘ
monoclonal light chain in serum or urine ⓘ |
| hasCause |
clonal plasma cell disorder
ⓘ
misfolded immunoglobulin light chains ⓘ monoclonal gammopathy ⓘ |
| hasClinicalFeature |
arrhythmias
ⓘ
constipation ⓘ diarrhea ⓘ fatigue ⓘ heart failure with preserved ejection fraction ⓘ hepatomegaly ⓘ macroglossia ⓘ malabsorption ⓘ nephrotic syndrome ⓘ orthostatic hypotension ⓘ periorbital purpura ⓘ peripheral neuropathy ⓘ proteinuria ⓘ restrictive cardiomyopathy ⓘ weight loss ⓘ |
| hasDiagnosticTest |
99mTc‑pyrophosphate or DPD scintigraphy to differentiate from ATTR amyloidosis
ⓘ
bone marrow biopsy ⓘ cardiac MRI ⓘ echocardiography ⓘ immunohistochemistry for light chains ⓘ mass spectrometry typing of amyloid ⓘ serum and urine protein electrophoresis ⓘ serum free light chain assay ⓘ tissue biopsy with Congo red staining ⓘ |
| hasEpidemiology |
more common in older adults
ⓘ
rare disorder with incidence of a few cases per million per year ⓘ |
| hasGoalOfTherapy |
hematologic complete response
ⓘ
organ response ⓘ |
| hasOutcome |
high mortality without treatment
ⓘ
progressive organ dysfunction ⓘ |
| hasPathophysiology |
amyloid fibril deposition in tissues
ⓘ
organ infiltration by amyloid ⓘ toxic effects of circulating light chains ⓘ |
| hasPrognosticFactor |
baseline cardiac biomarkers
ⓘ
cardiac involvement ⓘ difference between involved and uninvolved free light chains ⓘ |
| hasTreatment |
bortezomib-based chemotherapy
ⓘ
cyclophosphamide-bortezomib-dexamethasone regimen ⓘ daratumumab-based regimens ⓘ high-dose melphalan with autologous stem cell transplant ⓘ renal replacement therapy in kidney failure ⓘ supportive heart failure therapy ⓘ |
| isPreventedBy | no established primary prevention ⓘ |
How these facts were elicited
The pipeline generated the facts above by prompting gpt-5.1 with this entity's name + description and the instruction below.
Instruction
You are a knowledge base construction expert. Given a subject entity and a description of it, return factual statements that you know for the subject as a JSON list of dictionaries(triples), where keys must be "subject", "predicate" and "object". The number of facts may be very high, between 25 to 50 or more, for very popular subjects. For less popular subjects, the number of facts can be very low, like 5 or 10. # Requirements - If you don't know the subject at all, return an empty list. - If the subject is not a named entity, return an empty list. - Include at least one triple where predicate is "instanceOf". - Do not get too wordy. - Separate several objects into multiple triples with one object.
Input
Subject: light chain amyloidosis Description of subject: Light chain amyloidosis is a rare systemic disorder in which misfolded immunoglobulin light chains form amyloid deposits in organs such as the heart and kidneys, leading to progressive organ dysfunction.
Referenced by (1)
Full triples — surface form annotated when it differs from this entity's canonical label.