Splenic marginal zone lymphoma
Synonyms: SMZL
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
Splenic marginal zone lymphoma is a rare, indolent B-cell non-Hodgkin lymphoma characterized by abnormal clonal proliferation of mature B-lymphocytes with involvement in the spleen, bone marrow and, frequently, the blood. It usually presents with splenomegaly, lymphocytosis, anemia and/or thrombocytopenia. Hepatitis C virus and autoimmune manifestations, such as autoimmune hemolytic anemia and autoimmune thrombocytopenia, could be associated.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Splenic marginal zone lymphoma | SMZL |
| fr | Lymphome splénique de la zone marginale | SMZL · LSZM |
| de | Splenisches Marginalzonen-Lymphom | = en |
| es | Linfoma esplénico de la zona marginal | = en |
| it | Linfoma splenico della zona marginale | = en |
| nl | Splenisch marginale zone lymfoom | = en |
| pt | Linfoma esplénico da zona marginal | |
| pl | Chłoniak strefy brzeżnej śledziony | = en |
| cs | Splenický lymfom z marginální zóny | = en |
| uk | Лімфома маргінальної зони селезінки | = en |
| zh | 脾边缘区淋巴瘤 | = en |
| tr | Splenik marjinal bölge lenfoması | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C83.0 | NTBT | inclusion | validated |
| ICD-11 | 2A82.Y | NTBT | index | validated |
| ICD-11 | XH0MV1 | E | specific | validated |
| MONDO | 0019462 | E | validated | |
| UMLS | C0349632 | E | validated | |
| MedDRA | 10062113 | 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 / 1 000 000 | 0.5 / 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/86854
- This record as JSON: /data/d/86854.json