ORPHA:48686
Primary effusion lymphoma
Synonyms: Body cavity-based lymphoma · PEL
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult
- Status
- active
Primary effusion lymphoma (PEL) is a large B-cell lymphoma located in the body cavities, characterized by pleural, peritoneal, and pericardial fluid lymphomatous effusions and that is always associated with human herpes virus-8 (HHV-8).
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary effusion lymphoma | Body cavity-based lymphoma · PEL |
| fr | Lymphome primitif des séreuses | PEL · Lymphome des cavités |
| de | Primäres Effusionslymphom | PEL |
| es | Linfoma primario de efusiones | Linfoma primario de cavidades · PEL |
| it | Linfoma primitivo effusivo | Linfoma delle cavità corporee · PEL |
| nl | Primair effusielymfoom | Op lichaamsholte gebaseerd lymfoom · PEL |
| pt | Linfoma de derrame primário | PEL |
| pl | Pierwotny chłoniak wysiękowy | PEL |
| cs | Primární efuzní lymfom | PEL · Lymfom v tělních dutinách |
| uk | Первинна випотна лімфома | Лімфома на основі порожнин тіла · PEL |
| zh | 原发渗出性淋巴瘤 | 原发于体腔淋巴瘤 |
| tr | Primer efüzyon lenfoma | PEL · Vücut boşluğu temelli lenfoma |
Definition in:
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Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C83.8 | NTBT | inclusion | validated |
| ICD-11 | 2A81.9 | E | specific | validated |
| MONDO | 0018842 | E | validated | |
| MeSH | D054685 | E | validated | |
| UMLS | C1292753 | E | validated | |
| GARD | 9247 | E | validated | |
| MedDRA | 10065857 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (3):
- 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 - ORPHA:102024Human herpesvirus 8-related disorder
rare neoplastic diseases
No children.
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 200 cases | Worldwide | Case(s) | Validated | [EXPERT] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | 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.
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HPO phenotypes
8 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0003270 | Abdominal distention | |
| HP:0002027 | Abdominal pain | |
| HP:0002585 | Abnormality of the peritoneum | |
| HP:0012191 | B-cell lymphoma | |
| HP:0002094 | Dyspnea | |
| HP:0001698 | Pericardial effusion | |
| HP:0002202 | Pleural effusion | |
| Frequent (79-30%) | ||
| HP:0002721 | Immunodeficiency | |
Run the differential on the 7 obligate / very frequent terms
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/48686
- This record as JSON: /data/d/48686.json