Light and heavy chain deposition disease
Synonyms: LHCDD
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Status
- active
A rare non-amyloid monoclonal immunoglobulin deposition disease characterized by secretion of abnormal light and heavy chains, which are deposited in tissues and cause organ dysfunction, but do not form amyloid beta-pleated sheets or contain an amyloid P component. The condition most frequently occurs in association with multiple myeloma. The kidneys are most commonly affected (clinically manifesting as nephrotic syndrome and renal failure), but liver, heart, peripheral nerves, blood vessels, and joints may also be involved.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Light and heavy chain deposition disease | LHCDD |
| fr | Maladie de dépôt des chaînes légères et lourdes | |
| de | Leichtketten- und Schwerketten-Speicherkrankheit | = en |
| es | Enfermedad por depósito de cadenas pesadas y ligeras | |
| it | Malattia da deposito di catene leggere e pesanti | = en |
| nl | Lichte- en zwareketendepositieziekte | = en |
| pt | Doença de deposição de cadeias leves e pesadas | |
| pl | Choroba deponowania łańcuchów lekkich i ciężkich | = en |
| cs | Nemoc z depozice lehkých a těžkých řetězců | = en |
| uk | Хвороба відкладення легких та важких ланцюгів | = en |
| zh | 轻-重链沉积病 | = en |
| tr | Hafif ve ağır 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 | 2A83.51 | E | specific | validated |
| MONDO | 0019729 | E | validated | |
| UMLS | C5204117 | 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/93557
- This record as JSON: /data/d/93557.json