Epstein-Barr virus-positive diffuse large B-cell lymphoma
Synonyms: EBV-positive DLBCL · Epstein-Barr virus-positive diffuse large B-cell lymphoma not otherwise specified · Epstein-Barr virus-positive diffuse large B-cell lymphoma, NOS
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly is a rare form of diffuse large B-cell lymphoma occurring most commonly in patients over the age of 50 (usually between 70-75 years of age), without overt immunodeficiency, and presenting with nodal and extranodal involvement (in sites such as the stomach, lung, skin and pancreas) and B symptoms (fever, night sweats, weight loss). The tumor is characterized by an aggressive course and a short survival rate.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Epstein-Barr virus-positive diffuse large B-cell lymphoma | EBV-positive DLBCL · Epstein-Barr virus-positive diffuse large B-cell lymphoma not otherwise specified · Epstein-Barr virus-positive diffuse large B-cell lymphoma, NOS |
| fr | Lymphome B diffus à grandes cellules positif au virus Epstein-Barr | DLBCL positif à l'EBV · Lymphome B diffus à grandes cellules positif au virus Epstein-Barr, sans autre spécification |
| de | Epstein-Barr Virus-positives diffuses großzelliges B-Zell-Lymphom | EBV-positives DLBCL · Epstein-Barr-Virus-positives diffuses großzelliges B-Zell-Lymphom, nicht weiter spezifiziert |
| es | Linfoma difuso de células B grandes positivo para el virus de Epstein-Barr | LDCBG positivo para EBV · Linfoma difuso de células B grandes positivo para el virus de Epstein-Barr sin otra especificación |
| it | Linfoma diffuso a grandi cellule B positivo al virus di Epstein-Barr | DLBCL EBV-positivo · Linfoma diffuso a grandi cellule B positivo al virus di Epstein-Barr NAS · Linfoma diffuso a grandi cellule B positivo al virus di Epstein-Barr non altrimenti specificato |
| nl | Epstein-Barrvirus-positief diffuus grootcellig B-cellymfoom | EBV-positief DLBCL · Epstein-Barrvirus-positief diffuus grootcellig B-cellymfoom niet anderszins omschreven · Epstein-Barrvirus-positief diffuus grootcellig B-cellymfoom, NAO |
| pt | Linfoma B difuso de grandes células positivo para o vírus Epstein-Barr | DLBCL EBV-positivo · Linfoma B difuso de grandes células positivo para o vírus Epstein-Barr sem outra especificação · Linfoma B difuso de grandes células positivo para o vírus Epstein-Barr, NOS |
| pl | Chłoniak rozlany z dużych komórek B wieku podeszłego wirus Ebsteina-Barra-pozytywny | DLBCL wieku podeszłego EBV-pozytywny · Rozlany chłoniak z komórek B Epstein-Barr dodatni |
| cs | Difuzní velkobuněčný B-lymfom s pozitivitou viru Epsteina-Barrové s nástupem ve vyšším věku | EBV-pozitivní DLBCL ve stáří · Difuzní velkobuněčný B-lymfom s pozitivitou viru Epstein-Barrové s nástupem ve vyšším věku |
| uk | Епштейн-Барр-вірус-позитивна дифузна велико-В-клітинна лімфома | EBV-позитивний DLBCL · Вірусопозитивна дифузна велика В-клітинна лімфома з вірусом Епштейна-Барр, не вказано інше · Вірус-позитивна дифузна велика В-клітинна лімфома Епштейна-Барр, NOS |
| zh | 老年人EB病毒阳性弥漫性大B细胞淋巴瘤 | 老年人EB病毒阳性弥漫性大B细胞淋巴瘤 |
| tr | Epstein-Barr virüsü-yaşlılarda pozitif yaygın büyük B hücreli lenfoma | Yaşlıların EBV pozitif DLBCL'si |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C83.3 | NTBT | attributed | validated |
| ICD-11 | 2A81.6 | E | specific | validated |
| MONDO | 0017346 | E | validated | |
| UMLS | C2700007 | 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:544Diffuse large B-cell lymphoma
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders - ORPHA:289644Epstein-Barr virus-associated malignant lymphoproliferative disorder
rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | Unknown | — | Worldwide | Class only | Validated | WHO Classification of Tumours[OTHER] |
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | WHO Classification of Tumours[OTHER] |
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/289661
- This record as JSON: /data/d/289661.json