Unicentric Castleman disease
Synonyms: Localized Castleman disease
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Age of onset
- All ages
- Status
- active
A rare lymphoid hemopathy characterized by involvement of a single lymph node or a group of lymph nodes in one lymph node station (most commonly in the mediastinum, neck, abdomen, or retroperitoneum). Histopathologically, it may occur as a hyaline vascular subtype with hyaline-vascular follicles and a fibrotic and hypervascular stroma with sinus compression, or a mixed/plasmacytic subtype with dense, interfollicular sheets of (usually polytypic) plasma cells extending to the cortex, and variably sized lymphoid follicles including some with regressive changes. Clinically, most patients are asymptomatic, and lesions are detected incidentally.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Unicentric Castleman disease | Localized Castleman disease |
| fr | Maladie de Castleman unicentrique | Maladie de Castleman localisée |
| de | Castleman-Krankheit, unizentrische | Castleman-Krankheit, lokalisierte |
| es | Enfermedad de Castleman unicéntrica | Enfermedad de Castleman localizada |
| it | Malattia localizzata di Castleman | |
| nl | Unicentrische ziekte van Castleman | Gelokaliseerde ziekte van Castleman |
| pt | Doença Castleman unicêntrica | |
| pl | Zlokalizowana choroba Castlemana | = en |
| cs | Lokalizovaná Castlemanova choroba | = en |
| uk | Уніцентрична хвороба Катслмана | Локалізована хвороба Каслмана |
| zh | 单中心型Castleman病 | 局限性Castleman病 |
| tr | Unicentrik Castleman hastalığı | Lokalize Castleman hastalığı |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D47.7 | NTBT | attributed | validated |
| ICD-11 | 4B2Y | NTBT | index | validated |
| MONDO | 0019753 | E | validated | |
| UMLS | C3898582 | E | validated | |
| GARD | 6005 | 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:160Castleman disease
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
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET_29157611[PMID] |
| Annual incidence | 1-9 / 100 000 | — | United States | Class only | Validated | ORPHANET_25120049[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/93685
- This record as JSON: /data/d/93685.json