Splenic diffuse red pulp small B-cell lymphoma
Synonyms: SDRPL · Splenic diffuse red pulp lymphoma
- Type
- Disease
- Group
- Disorder
- Status
- active
Splenic diffuse red pulp small B-cell lymphoma is a rare, indolent B-cell non-Hodgkin lymphoma characterized by abnormal proliferation of small, monomorphous, basophilic B-lymphocytes, with villous cytoplasm, in the splenic red pulp, bone marrow and peripheral blood. It typically presents in the late clinical stages with splenomegaly and moderate lymphocytosis. Cytopenias are rare and likely associated with hypersplenism.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Splenic diffuse red pulp small B-cell lymphoma | SDRPL · Splenic diffuse red pulp lymphoma |
| fr | Lymphome splénique diffus de la pulpe rouge à petites cellules B | SDRPL |
| de | B-Zell-Lymphom der roten Pulpa, diffuses splenisches | SDRPL · Splenisches diffuses kleinzelliges B-Zell-Lymphom der roten Pulpa |
| es | Linfoma esplénico de células B con infiltración difusa de la pulpa roja | Linfoma esplénico difuso de la pulpa roja · SDRPL |
| it | Linfoma splenico diffuso della polpa rossa | Linfoma B splenico diffuso della polpa rossa · SDRPL |
| nl | Splenisch diffuus kleincellig B-cellymfoom van rode pulpa | Diffuus kleincellig B-cellymfoom van rode pulpa van milt · SDRPL · Splenisch diffuus lymfoom van rode pulpa |
| pt | Linfoma difuso da polpa vermelha do baço | |
| pl | Chłoniak rozlany miazgi czerwonej śledziony z małych komórek B | SDRpl · Chłoniak rozlany miazgi czerwonej śledziony |
| cs | Difuzní lymfom červené dřeně sleziny | SDRPL · Slezinný difuzní B-buněčný lymfom z červené dřeně |
| uk | Селезінкова дифузна червона пульпозна мала В-клітинна лімфома | SDRPL · Дифузна червона пульпозна лімфома селезінки |
| zh | 脾弥漫性红髓小B细胞淋巴瘤 | 脾弥漫性红髓淋巴瘤 |
| tr | Dalak difüz kırmızı pulpa küçük B-hücreli lenfoma | SDRPL · Dalak yaygın kırmızı pulpa lenfoması |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C83.0 | NTBT | attributed | validated |
| ICD-11 | 2A82.Y | NTBT | index | validated |
| ICD-11 | XH99V9 | E | specific | validated |
| MONDO | 0017599 | E | validated | |
| UMLS | C2699508 | 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
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/300869
- This record as JSON: /data/d/300869.json