Hepatosplenic T-cell lymphoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult
- Status
- active
A rare T-cell non-Hodgkin lymphoma characterized by a proliferation of cytotoxic T-cells, usually gamma delta T-cells, with involvement of the liver and spleen, but without involvement of lymph nodes. The bone marrow is consistently affected. Patients typically present during adolescence or young adulthood with hepatosplenomegaly, pancytopenia, and systemic symptoms. Peripheral blood involvement may develop later in the disease course. There is a clear male preponderance. The disease often occurs in the context of long-term immunosuppression. The course is aggressive with poor therapy response.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Hepatosplenic T-cell lymphoma | |
| fr | Lymphome T hépatosplénique | |
| de | T-Zell-Lymphom, hepatosplenisches | |
| es | Linfoma hepatoesplénico de células T | |
| it | Linfoma epatosplenico a cellule T | |
| nl | Hepatosplenisch T-cellymfoom | |
| pt | Linfoma hepatoesplénico de células T | |
| pl | Wątrobowo-śledzionowy chłoniak T-komórkowy | |
| cs | Hepatosplenický T-lymfom | |
| uk | Гепатоспленоцитарна Т-клітинна лімфома | |
| zh | 肝脾T细胞淋巴瘤 | |
| tr | Hepatosplenik T-hücreli lenfoma |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C86.1 | E | specific | validated |
| ICD-11 | 2A90.8 | E | specific | validated |
| MONDO | 0019474 | E | validated | |
| UMLS | C1333984 | E | validated | |
| MedDRA | 10066957 | 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:171918T-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
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.03 / 100 000 | United States | Value and class | Validated | 21823196[PMID] |
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet 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/86882
- This record as JSON: /data/d/86882.json