Hydroa vacciniforme-like lymphoma
Synonyms: Angiocentric cutaneous T-cell lymphoma of childhood · HVLL · Hydroa-like cutaneous T-cell lymphoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Childhood
- Status
- active
A very rare Epstein-Barr virus-associated lymphoproliferative disorder characterized by a chronic, recurrent, vesiculopapular rash, which subsequently ulcerates and scars, located mainly on sun-exposed areas and which is associated with systemic manifestations, such as fever, weight loss, asthenia, facial edema, arthralgia, lymphadenopathy, hepatosplenomegaly and/or increased liver enzymes. Hypersensitivity to mosquito bites has been associated and an increased risk of developing systemic lymphoma has been reported.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Hydroa vacciniforme-like lymphoma | Angiocentric cutaneous T-cell lymphoma of childhood · HVLL · Hydroa-like cutaneous T-cell lymphoma |
| fr | Lymphome à type d'hydroa vacciniforme | |
| de | Hydroa vacciniforme-ähnliche lymphoproliferative Erkrankung | Hydroa vacciniforme-artige lymphoproliferative Krankheit · Hydroa-artiges kutanes T-Zell-Lymphom · Angiozentrisches kutanes T-Zell-Lymphom der Kindheit · HV-LPD |
| es | Linfoma similar a hidroa vacciniforme | HVLL · Linfoma cutáneo de células T angiocéntrico de la infancia · Linfoma cutáneo de células T similar a hidroa · Linfoma de hidroa vacciniforme-like |
| it | Linfoma idroa vacciniforme-simile | |
| nl | Hydroa vacciniforme-achtig lymfoom | Angiocentrisch cutaan T-cellymfoom in de kindertijd · HVLL · Hydroa-achtig cutaan T-cellymfoom |
| pt | Linfoma semelhante a Hidroa vaciniforme | |
| pl | Chłoniak typu opryszczki ospówkowatej | HVLL |
| cs | Lymfom "Hydroa vacciniforme-like | = en |
| uk | Hydroa vacciniforme-подібна лімфома | Ангіоцентрична шкірна Т-клітинна лімфома дитячого віку · HVLL · Гідроа-подібна шкірна Т-клітинна лімфома |
| zh | 牛痘水疱样淋巴瘤 | 儿童血管中心皮肤T细胞淋巴瘤 |
| tr | Hydroa vacciniforme benzeri lenfoma | HVLL · Hidroa-benzeri kutanöz T-hücreli lenfoma · Çocukluk çağı anjiyosentrik kutanöz T-hücreli lenfoma |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C84.5 | NTBT | attributed | validated |
| ICD-11 | 2B0Y | NTBT | index | validated |
| MONDO | 0018224 | E | validated | |
| UMLS | C1708397 | 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:289644Epstein-Barr virus-associated malignant lymphoproliferative disorder
rare neoplastic diseases - ORPHA:171918T-cell non-Hodgkin lymphoma
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/364039
- This record as JSON: /data/d/364039.json