Primary lymphoma of the conjunctiva
Synonyms: Primary lymphoid conjunctival tumor
- Type
- Disease
- Group
- Disorder
- Status
- active
Primary lymphoma of the conjunctiva is an extremely rare clonal lymphoid proliferation of the ocular surface, with an indolent course. Clinically it presents with treatment-resistant conjunctivitis, ptosis, excessive tear production or as a painless, salmon-pink, ''fleshy'' patch, with a smooth or multinodular surface, on the bulbar conjunctiva. Histologically it is usually B-cell Non-Hodgkin lymphoma (most often extranodal marginal zone B-cell lymphoma, followed by follicular and diffuse large B-cell lymphoma), with conjunctival T-cell Non-Hodgkin lymphoma being very rare.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary lymphoma of the conjunctiva | Primary lymphoid conjunctival tumor |
| fr | Lymphome primitif de la conjonctive | |
| de | Bindehaut-Lymphom, primäres | Primärer lymphoider Tumor der Bindehaut |
| es | Linfoma primario de la conjuntiva | Tumor linfoide primario de la conjuntiva |
| it | Linfoma primitivo della congiuntiva | Tumore congiuntivale linfoide primitivo |
| nl | Primair lymfoom van conjunctiva | Primair lymfoom van bindvlies · Primaire lymfoïde conjunctivale tumor |
| pt | Linfoma primário da conjuntiva | |
| pl | Pierwotny chłoniak spojówki | Pierwotny guz limfoidalny rogówki |
| cs | Primární lymfom spojivky | Primární lymfoidní nádor spojivky |
| uk | Первинна лімфома конюнктиви | Первинна лімфоїдна пухлина конюнктиви |
| zh | 原发性结膜淋巴瘤 | = en |
| tr | Konjonktivanın primer lenfoması | Primer lenfoid konjonktival tümör |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C85.7 | NTBT | attributed | validated |
| ICD-11 | 2B33.5 | NTBT | attributed | validated |
| MONDO | 0017907 | E | validated | |
| UMLS | C4706485 | 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 (1):
- ORPHA:279911Primary organ-specific 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
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/319667
- This record as JSON: /data/d/319667.json