Indeterminate cell histiocytosis
Synonyms: Indeterminate dendritic cell neoplasm · Indeterminate dendritic cell tumor
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
A rare neoplastic disease characterized by multiple, and on occasion single, asymptomatic, smooth, red-brown papulonodules located on the face, neck, trunk and/or extremities which present a nonepidermotrophic histiocytic infiltrate with immunohistochemical features of both Langerhans and non-Langerhans cells (i.e. immunopositive for S100 protein and CD1a in the absence of Birbeck granules and langerin expression).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Indeterminate cell histiocytosis | Indeterminate dendritic cell neoplasm · Indeterminate dendritic cell tumor |
| fr | Histiocytose à cellule indeterminée | |
| de | Histiozytose der indeterminierten Zelle | Indeterminante dendritische Zellneubildung · Indeterminanter dendritischer Zelltumor |
| es | Histiocitosis de células indeterminadas | Neoplasia indeterminada de células dendríticas · Tumor indeterminado de células dendríticas |
| it | Istiocitosi a cellule indeterminate | |
| nl | Onbepaalde celhistiocytose | Onbepaald dendritische cel neoplasma · Onbepaalde dendritische celtumor |
| pt | Histiocitose de células não-determinadas | |
| pl | Histiocytoza z komórek nieokreślonych | = en |
| cs | Histiocytóza z neurčených buněk | = en |
| uk | Невизначений гістіоцитоз клітин | Невизначене новоутворення дендритних клітин · Невизначена дендритноклітинна пухлина |
| zh | 不确定细胞组织细胞增多症 | 不明原因树突状细胞瘤 |
| tr | Belirsiz hücreli histiyositoz | Belirsiz dendritik hücre tümörü · Belirsiz dendritik hücreli neoplazm |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C96.4 | NTBT | attributed | validated |
| ICD-11 | 2B31.6 | E | specific | validated |
| MONDO | 0015538 | E | validated | |
| UMLS | C2825741 | 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 (2):
- ORPHA:98289Dendritic cell tumor
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders - ORPHA:157987Non-Langerhans cell histiocytosis
rare skin diseases; rare systemic and rheumatological diseases
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/158019
- This record as JSON: /data/d/158019.json