Plasmablastic lymphoma
Synonyms: PBL
- Type
- Disease
- Group
- Disorder
- Status
- active
A rare aggressive B-cell non-Hodgkin lymphoma characterized by neoplastic cells resembling B immunoblasts or plasmablasts with a CD20-negative plasmacytic phenotype. The tumor may occur in the oral cavity, the gastrointestinal tract, or other, predominantly extranodal, sites and is typically associated with immunodeficiency or -suppression. The tumor cells are EBV-positive in most cases. Patients often present with disseminated bone involvement. Paraproteinemia may also be detected. Prognosis is generally poor.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Plasmablastic lymphoma | PBL |
| fr | Lymphome plasmoblastique | = en |
| de | Lymphom, plasmoblastisches | = en |
| es | Linfoma plasmablástico | = en |
| it | Linfoma plasmoblastico | = en |
| nl | Plasmablastisch lymfoom | = en |
| pt | Linfoma plasmablástico | = en |
| pl | Chłoniak plazmablastyczny | = en |
| cs | Plazmablastický lymfom | = en |
| uk | Плазмоцитарна лімфома | = en |
| zh | 浆母细胞淋巴瘤 | 浆母细胞淋巴瘤 |
| tr | Plazmablastik lenfoma | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C83.3 | NTBT | attributed | validated |
| ICD-11 | 2A81.2 | E | specific | validated |
| MONDO | 0017347 | E | validated | |
| MeSH | D000069293 | E | validated | |
| UMLS | C3472614 | E | validated | |
| GARD | 12125 | E | validated | |
| MedDRA | 10065039 | 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:300846Aggressive B-cell non-Hodgkin 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
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/289666
- This record as JSON: /data/d/289666.json