Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood
Synonyms: Systemic EBV+ T-cell LPD of childhood · Systemic EBV-positive T-cell lymphoproliferative disease of childhood
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Childhood
- Status
- active
A rare and very aggressive neoplastic disease emerging after a primary acute or chronic active EBV infection. It presents with persisting fever and malaise, hepatosplenomegaly with or without lymphadenopathy, liver failure, severe pancytopenia and a rapid progression towards multi-organ failure and hemophagocytic syndrome with a fatal issue. It is characterized by clonal proliferation of EBV-infected T cells with an activated cytotoxic phenotype.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood | Systemic EBV+ T-cell LPD of childhood · Systemic EBV-positive T-cell lymphoproliferative disease of childhood |
| fr | Maladie lymphoproliférative systémique à cellules T positive au virus Epstein-Barr chez l'enfant | Maladie lymphoproliférative systémique à cellules T positive à l'EBV chez l'enfant |
| de | Systemische EBV-positive T-Zell-lymphoproliferative Erkrankung der Kindheit | Systemische EBV+ T-Zell LPD der Kindheit |
| es | Enfermedad linfoproliferativa sistémica de células T asociada al virus Epstein-Barr de la infancia | ELP sistémica de células T EBV-positiva de la infancia · Enfermedad linfoproliferativa sistémica de células T EBV-positivas de la infancia |
| it | Malattia linfoproliferativa delle cellule T Epstein-Barr virus-positiva sistemica dell'infanzia | LPD delle cellule T EBV+ sistemica dell'infanzia · Malattia linfoproliferativa delle cellule T EBV-positiva sistemica dell'infanzia |
| nl | Systemische Epstein-Barrvirus-positieve T-cel lymfoproliferatieve ziekte van de kindertijd | Systemische EBV+ T-cel LPD van de kindertijd · Systemische EBV-positieve T-cel lymfoproliferatieve ziekte van de kindertijd |
| pt | Doença linfoproliferativa da infância com células T positivas para vírus Epstein-Barr sistémico | |
| pl | Układowa choroba limfoproliferacyjna z komórek T wirus Ebsteia i Barra pozytywna wieku dzieciecęgo | Układowa choroba limfoproliferacyjna z komórek T EBV pozytywna wieku dzieciecęgo · Układowa LPD z komórek T EBV+ wieku dziecięcego |
| cs | Systémová T-buněčná lymfoproliferativní nemoc dětského věku s pozitivitou viru Epsteina-Barrové | Systémová EBV+ T-buněčná LPD v dětství |
| uk | Системна Епштейн-Барр-вірус-позитивна Т-клітинна лімфопроліферативна хвороба дитячого віку | Системна ЕБВ+ Т-клітинна ЛПЗ дитячого віку · Системна ЕБВ-позитивна Т-клітинна лімфопроліферативна хвороба дитячого віку |
| zh | 儿童系统性EB病毒阳性-T淋巴细胞增生性疾病 | 儿童系统性EBV阳性T细胞淋巴增生性疾病 |
| tr | Çocukluk çağı sistemik Epstein-Barr virüsü pozitif T hücreli lenfoproliferatif hastalık | Çocukluk çağı sistemik EBV + T-hücreli LPD'si · Çocukluk çağı sistemik EBV pozitif T-hücreli lenfoproliferatif hastalığı |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D82.3 | NTBT | attributed | validated |
| MONDO | 0018223 | E | validated | |
| UMLS | C2699747 | 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
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Europe | Class only | Not yet validated | ORPHANET |
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/364033
- This record as JSON: /data/d/364033.json