ORPHA:86872
T-cell large granular lymphocyte leukemia
Synonyms: Proliferation of large granular lymphocytes · T-LGL · T-cell LGL leukemia
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
T-cell large granular lymphocyte leukemia (T-cell LGL leukemia) is a lymphoproliferative malignancy that arises from the mature T-cell (CD3+) lineage.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | T-cell large granular lymphocyte leukemia | Proliferation of large granular lymphocytes · T-LGL · T-cell LGL leukemia |
| fr | Leucémie à grands lymphocytes granuleux T | Leucémie à GLG T · Leucémie à LGL T · Prolifération de grands lymphocytes granuleux · T-GLG · T-LGL |
| de | T-Zell-Leukämie mit großen granulären Lymphozyten | T-LGL · T-LGL-Leukämie · T-Zell-LGL-Leukämie |
| es | Leucemia linfocítica granular de células T grandes | Leucemia LGL de células T · Proliferación de linfocitos granulares grandes · T-LGL |
| it | Leucemia a grandi linfocitici granulari T | Leucemia LGL a cellule T · Leucemia linfocitica cronica a cellule T · Proliferazione dei grandi linfociti granulari · T-LGL |
| nl | T-celleukemie met grote granulaire lymfocyten | Proliferatie van grote granulaire lymfocyten · T-LGL · T-cel LGL leukemie |
| pt | Leucemia linfocítica granular grande de células T | |
| pl | Białaczka z dużych ziarnistych limfocytów T | Proliferacja dużych ziarnistych limfocytów · T-cell LGL białaczka · T-LGL |
| cs | T-velkobuněčná granulární lymfocytární leukémie | T-LGL · Proliferace velkých granulárních lymfocytů · T-buněčná LGL leukémie |
| uk | Т-клітинна велика гранулярна лімфоцитарна лейкемія | Проліферація великих гранулярних лімфоцитів · Т-клітинна лейкемія LGL · T-LGL |
| zh | T细胞大颗粒淋巴细胞性白血病 | 大颗粒淋巴细胞增生 |
| tr | T-hücreli büyük granüler lenfosit lösemi | T-LGL · T hücreli LGL lösemi · Büyük granüler lenfositlerin çoğalması |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C91.7 | NTBT | inclusion | validated |
| ICD-11 | 2A90.1 | E | specific | validated |
| MONDO | 0019469 | E | validated | |
| UMLS | C1955861 | E | validated | |
| GARD | 9812 | E | validated | |
| MedDRA | 10065862 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:178996Acquired neutropenia
rare immunological diseases - ORPHA:512034Large granular lymphocyte leukemia
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
No children.
Codes that Orphanet moved or referred to this one:
- ORPHA:2687Neutropenia-hyperlymphocytosis with large granular lymphocytes syndrome moved to · inactive
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Genes
1 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| STAT3 | Disease-causing somatic mutation(s) in | Assessed | HGNC:11364 | ENSG00000168610 | 102582 | 17q21.2 | signal transducer and activator of transcription 3 | 29385279[PMID]_26980727[PMID] |
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Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.4 / 100 000 | Europe | Value and class | Validated | [EXPERT] |
| 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.
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HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/86872
- This record as JSON: /data/d/86872.json