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.
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 ⓘ |
Referenced by (1)
Full triples — surface form annotated when it differs from this entity's canonical label.