Primary pulmonary lymphoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A rare neoplastic disease defined as a clonal lymphoid proliferation affecting one or both lungs (parenchyma and/or bronchi) in a patient with no detectable extrapulmonary involvement at diagnosis or during the subsequent 3 months. PPL comprises low grade/indolent B cell PPL forms, the most frequent form represented by the marginal B-cell lymphoma of mucosa associated lymphoid tissue (MALT lymphoma) and other non-MALT low grade lymphomas; and more rarely high-grade B-cell PPL (including diffuse large B cell lymphoma) and lymphomatoid granulomatosis (LYG).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary pulmonary lymphoma | |
| fr | Lymphome pulmonaire primitif | |
| de | Lungenlymphom, primäres | |
| es | Linfoma pulmonar primario | |
| it | Linfoma polmonare primitivo | |
| nl | Primair pulmonaal lymfoom | |
| pt | Linfoma pulmonar primário | |
| pl | Pierwotny chłoniak płuc | |
| cs | Primární plicní lymfom | |
| uk | Первинна лімфома легень | |
| zh | 原发性肺淋巴瘤 | |
| tr | Primer pulmoner lenfoma |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C85.7 | NTBT | attributed | validated |
| ICD-11 | 2C25.Y | NTBT | index | validated |
| MONDO | 0016570 | E | validated | |
| UMLS | C4273669 | E | validated | |
| MedDRA | 10037418 | 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:279911Primary organ-specific lymphoma
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders - ORPHA:101945Rare bronchopulmonary and pleural cavity tumors
rare respiratory diseases; rare neoplastic diseases
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 | — | Worldwide | 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/2420
- This record as JSON: /data/d/2420.json