Lymphomatoid granulomatosis
Synonyms: LYG
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
Lymphomatoid granulomatosis (LYG) is a very rare Epstein-Barr virus (EBV)-driven lymphoproliferative disease most commonly occurring in adults (in the fourth to sixth decade of life) and commonly affecting the lungs (with presentations varying from small bilateral pulmonary nodules to large necrotic and sometimes cavitating lesions), skin, central nervous system, and kidneys, but only very rarely affecting the lymph nodes and spleen. The symptoms associated with LYG depend on the site of disease involvement but mainly include cough, dyspnea or chest pain (in those with pulmonary involvement) and constitutional symptoms such as weight loss and fever.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Lymphomatoid granulomatosis | LYG |
| fr | Granulomatose lymphomatoïde | = en |
| de | Lymphomatoide Granulomatose | = en |
| es | Granulomatosis linfomatoide | = en |
| it | Granulomatosi linfomatoide | = en |
| nl | Lymfomatoïde granulomatose | = en |
| pt | Granulomatose linfomatóide | |
| pl | Ziarniniakowatość limfoidalna (nazwa polska jest myląca, gdyż wskazuje na zmianę łagodną co nie jest prawdą i dlatego lepiej nie tłumaczyć nazwy anglojęzycznej) | = en |
| cs | Lymfomatoidní granulomatóza | = en |
| uk | Лімфоматоїдний гранулематоз | = en |
| zh | 淋巴瘤样肉芽肿 | = en |
| tr | Lenfomatoid granülomatoz | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C83.8 | NTBT | inclusion | validated |
| ICD-11 | 2A81.3 | E | specific | validated |
| MONDO | 0019466 | E | validated | |
| MeSH | D008230 | E | validated | |
| UMLS | C0024307 | E | validated | |
| GARD | 6943 | E | validated | |
| MedDRA | 10025325 | 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
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | 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.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/86869
- This record as JSON: /data/d/86869.json