ORPHA:86864
Heavy chain disease
Synonyms: HCD
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult
- Status
- active
Heavy-chain diseases (HCDs) are rare monoclonal lymphoplasma-cell proliferative disorders involving B cells and are characterized by the synthesis of truncated heavy chains without associated light chains.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Heavy chain disease | HCD |
| fr | Maladie des chaînes lourdes | = en |
| de | Schwerketten-Krankheit | = en |
| es | Enfermedad de las cadenas pesadas | ECP · HCD |
| it | Malattia delle catene pesanti | = en |
| nl | Zwareketenziekte | = en |
| pt | Doenças de cadeias pesadas | |
| pl | Choroba łańcuchów ciężkich | = en |
| cs | Nemoc těžkých řetězců | = en |
| uk | Хвороба важких ланцюгів | = en |
| zh | 重链病 | = en |
| tr | Ağır zincir hastalığı | = en |
Definition in:
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Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C88.2 | BTNT | specific | validated |
| ICD-10 | C88.3 | BTNT | specific | validated |
| ICD-11 | 2A84 | E | specific | validated |
| MONDO | 0019464 | E | validated | |
| MeSH | D006362 | E | validated | |
| UMLS | C0018852 | E | validated | |
| MedDRA | 10019350 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:98282Plasma cell tumor
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
| Annual incidence | <1 / 1 000 000 | — | Europe | Class only | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
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HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/86864
- This record as JSON: /data/d/86864.json