Light chain deposition disease
Synonyms: LCDD
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Status
- active
A rare non-amyloid monoclonal immunoglobulin deposition disease characterized by deposition of abnormal immunoglobulin light chains in the kidneys, resulting in nephrotic syndrome and renal failure. Symptomatic extrarenal deposition is uncommon, although hepatic, cardiac, and neural deposits have been reported. The condition frequently occurs in association with multiple myeloma or in patients with M protein and marrow plasma cells at monoclonal gammopathy of undetermined significance levels.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Light chain deposition disease | LCDD |
| fr | Maladie de dépôt des chaînes légères | = en |
| de | Leichtketten-Speicherkrankheit | = en |
| es | Enfermedad por depósito de cadenas ligeras | |
| it | Malattia da deposito di catene leggere | = en |
| nl | Lichteketendepositieziekte | = en |
| pt | Doença de deposição de cadeias leves | |
| pl | Choroba deponowania łańcuchów lekkich | = en |
| cs | Nemoc z depozit lehkých řetězců | = en |
| uk | Хвороба відкладення легких ланцюгів | = en |
| zh | 轻链沉积病 | = en |
| tr | Hafif zincir birikimi hastalığı | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D89.8 | NTBT | attributed | validated |
| ICD-11 | 2A83.52 | E | specific | validated |
| MONDO | 0019730 | E | validated | |
| UMLS | C0238239 | E | validated | |
| GARD | 6906 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:86861Non-amyloid monoclonal immunoglobulin deposition disease
rare renal diseases; rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/93558
- This record as JSON: /data/d/93558.json