Lymphoplasmacytic lymphoma without IgM production
Synonyms: Lymphoplasmacytic lymphoma without Immunoglobulin M production
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
A rare B-cell non-Hodgkin lymphoma characterized by the presence of small B-lymphocytes, plasmacytoid lymphocytes, and plasma cells, and either non-secreting or secreting IgG or IgA paraproteins. The disease usually involves the bone marrow, sometimes also the spleen or lymph nodes. Patients typically present with symptoms related to anemia. Hyperviscosity, autoimmune phenomena, and B symptoms may also be observed. Mortality is higher as compared to Waldenström macroglobulinemia.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Lymphoplasmacytic lymphoma without IgM production | Lymphoplasmacytic lymphoma without Immunoglobulin M production |
| fr | Lymphome lymphoplasmocytaire sans sécrétion d'IgM | Lymphome lymphoplasmocytaire sans sécrétion d'immunoglobuline M |
| de | Lymphoplasmozytisches Lymphom ohne IgM-Produktion | |
| es | Linfoma linfoplasmacítico sin producción de IgM | |
| it | Linfoma linfoplasmocitico senza produzione di IgM | |
| nl | Lymfoplasmacytair lymfoom zonder IgM-productie | |
| pt | Linfoma linfoplasmocítico sem produção de IgM | |
| pl | Chłoniak limfoplazmatyczny bez produkcji IgM | = en |
| cs | Lymfoplazmocytární lymfom bez produkce IgM | Lymfoplazmocytární lymfom bez produkce imunoglobulinu M |
| uk | Лімфоплазмоцитарна лімфома без вироблення IgM | Лімфоплазмоцитарна лімфома без вироблення імуноглобуліну М |
| zh | 不产IgM的淋巴浆细胞性淋巴瘤 | 不产IgM的淋巴浆细胞性淋巴瘤 |
| tr | IgM üretimi olmayan lenfoplazmasitik lenfoma | İmmünoglobulin M üretimi olmayan lenfoplazmasitik lenfoma |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C83.0 | NTBT | attributed | validated |
| MONDO | 0018621 | E | validated | |
| UMLS | C5680061 | 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:300842Indolent B-cell non-Hodgkin 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/443159
- This record as JSON: /data/d/443159.json