T-cell prolymphocytic leukemia
Synonyms: T-PLL · T-cell chronic lymphocytic leukemia
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A rare mature T-cell neoplasm characterized by proliferation of small to medium-sized prolymphocytes with a mature post-thymic T-cell phenotype, involving the peripheral blood, bone marrow, lymph nodes, liver, spleen, and sometimes the skin. T-cell receptor genes are clonally rearranged. Patients typically present with hepatosplenomegaly, generalized lymphadenopathy, high leukocyte count with normal serum immunoglobulins, anemia, and thrombocytopenia. HTLV-1 serology is negative. The disease course is aggressive with generally poor prognosis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | T-cell prolymphocytic leukemia | T-PLL · T-cell chronic lymphocytic leukemia |
| fr | Leucémie prolymphocytaire à cellules T | LPL-T · Leucémie lymphocytaire T chronique · T-PLL |
| de | T-Zell-Prolymphozytenleukämie | Prolymphozytenleukämie vom T-Zell-Typ · T-PLL |
| es | Leucemia prolinfocítica de células T | Leucemia linfocítica crónica de células T · T-PLL |
| it | Leucemia prolinfocitica a cellule T | Leucemia linfocitica cronica a cellule T · T-PLL |
| nl | T-cel-prolymfocytenleukemie | T-PLL · T-cel chronische lymfocytaire leukemie |
| pt | Leucemia prolinfocítica de células T | |
| pl | Białaczka prolimfocytowa T-komórkowa | Przewlekła białaczka limfocytarna z komórek T · T-plL |
| cs | T-prolymfocytární leukémie | T-PLL · Chronická T-lymfocytární leukémie |
| uk | Т-клітинна пролімфоцитарна лейкемія | Т-клітинна хронічна лімфоцитарна лейкемія · T-PLL |
| zh | T细胞幼淋巴细胞白血病 | T细胞慢性淋巴细胞白血病 |
| tr | T-hücreli prolenfositik lösemi | T-PLL · T hücreli kronik lenfositik lösemi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C91.6 | E | specific | validated |
| ICD-11 | 2A90.0 | E | specific | validated |
| MONDO | 0019468 | E | validated | |
| MeSH | D015461 | E | validated | |
| UMLS | C2363142 | E | validated | |
| GARD | 13731 | E | validated | |
| MedDRA | 10042985 | 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 | Unknown | — | Worldwide | Class only | Validated | WHO Classification of Tumours[OTHER] |
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | WHO Classification of Tumours[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/86871
- This record as JSON: /data/d/86871.json