Lymphoplasmacytic inflammatory pseudotumor of the liver
Synonyms: IgG4-related inflammatory pseudotumor of the liver
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A subtype of inflammatory pseudotumor of the liver characterized by a benign, well-circumscribed tumor with diffuse lymphoplasmacytic infiltration with histological features of IgG4-related disease (numerous IgG4-positive plasma cells, prominent eosinophils, stromal fibrosis, fibroblastic proliferations and, frequently, obliterative phlebitis), and that is likely located around the hepatic hilum. Most often it is discovered as an incidental finding.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Lymphoplasmacytic inflammatory pseudotumor of the liver | IgG4-related inflammatory pseudotumor of the liver |
| fr | Pseudotumeur inflammatoire du foie, forme lymphoplasmocytaire | Pseudotumeur inflammatoire du foie associée à IgG4 |
| de | Lymphoplasmazytischer inflammatorischer Pseudotumor der Leber | IgG4-assoziierter inflammatorischer Pseudotumor der Leber |
| es | Pseudotumor inflamatorio linfoplasmocítico del hígado | Pseudotumor inflamatorio del hígado asociado a IgG4 · Pseudotumor inflamatorio hepático linfoplasmocítico |
| it | Pseudotumore infiammatorio linfoplasmatico del fegato | |
| nl | Lymfoplasmacytaire inflammatoire pseudotumor van lever | IgG4-gerelateerde inflammatoire pseudotumor van lever |
| pt | Pseudotumor inflamatório linfoplasmocítico do fígado | |
| pl | Guz rzekomy limfoplazmatyczny zapalny wątroby | Guz rzekomy limfoplazmatyczny zapalny wątroby związany z IgG4 |
| cs | Lymfoplazmocytický zánětlivý pseudotumor jater | IgG4-vázaný zánětlivý pseudotumor jater |
| uk | Лімфоплазмоцитарна запальна псевдопухлина печінки | Запальна псевдопухлина печінки, повязана з IgG4 |
| zh | 肝淋巴浆细胞性炎性假肿瘤 | IgG4相关肝脏炎症性假瘤 |
| tr | Karaciğer lenfoplazmasitik inflamatuar psödotümörü | Karaciğerin IgG4 ile ilişkili inflamatuar psödotümörü |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K75.8 | NTBT | attributed | validated |
| MONDO | 0035313 | E | validated | |
| UMLS | C5680170 | 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:284264IgG4-related disease
rare systemic and rheumatological diseases - ORPHA:90003Inflammatory pseudotumor of the liver
rare hepatic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET_17571078[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/555437
- This record as JSON: /data/d/555437.json