Nodal marginal zone B-cell lymphoma
Synonyms: NMZL
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Elderly
- Status
- active
Nodal marginal zone B-cell lymphoma is a rare, indolent B-cell non-Hodgkin lymphoma, characterized by abnormal clonal proliferation of mature B-lymphocytes with involvement of the lymph nodes, sometimes the bone marrow, and rarely the blood. Clinically it presents with disseminated peripheral, abdominal and/or thoracic lymphadenopathy. Cytopenia and bulky tumors (greater than 5 cm) are rare. Association with Hepatitis C virus and chronic inflammation has been reported.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Nodal marginal zone B-cell lymphoma | NMZL |
| fr | Lymphome nodulaire à cellules B de la zone marginale | LNZM · NMZL |
| de | Nodales Marginalzonen-B-Zell-Lymphom | = en |
| es | Linfoma nodal de zona marginal de células B | = en |
| it | Linfoma nodulare a cellule B della zona marginale | = en |
| nl | Nodaal marginale zone B-cellymfoom | = en |
| pt | Linfoma nodal de células B da zona marginal | |
| pl | Węzłowy chłoniak strefy brzeżnej B-komórkowy | = en |
| cs | Uzlinový B-buněčný lymfom z marginální zóny | = en |
| uk | Вузлова маргінальна В-клітинна лімфома | = en |
| zh | 淋巴结边缘区B细胞淋巴瘤 | = en |
| tr | Nodal marjinal bölge B-hücreli lenfoma | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C83.0 | NTBT | inclusion | validated |
| ICD-11 | 2A85.0 | E | specific | validated |
| MONDO | 0019465 | E | validated | |
| UMLS | C0855139 | E | validated | |
| MedDRA | 10029460 | 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:300912Marginal zone 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
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 100 000 | 1 / 100 000 | Europe | Value and class | Validated | European Medecines Agency 2013[OTHER] |
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/86867
- This record as JSON: /data/d/86867.json