Aggressive NK-cell leukemia
Synonyms: ANKCL · Aggressive NK-cell lymphoma · NK-cell LGL leukemia · NK-cell large granular lymphocyte leukemia
- Type
- Disease
- Group
- Disorder
- Inheritance
- Multigenic/multifactorial, Not applicable
- Age of onset
- Adult
- Status
- active
An extremely rare and highly aggressive neoplasm, usually manifesting in the third to fourth decade of life, affecting males and females equally, and characterized by the onset of high fever, weight loss, jaundice, skin infiltration, lymphadenopathy, hepatosplenomegaly, and severe anemia. It has a fulminant and rapidly fatal disease course with the progressive appearance of multiorgan failure and disseminated intravascular coagulation.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Aggressive NK-cell leukemia | ANKCL · Aggressive NK-cell lymphoma · NK-cell LGL leukemia · NK-cell large granular lymphocyte leukemia |
| fr | Leucémie agressive à cellules NK | ANKCL · Leucémie à GLG NK · Leucémie à LGL NK · Leucémie à grands lymphocytes granuleux NK |
| de | Agressive NK-Zell-Leukämie | ANKCL · NK-Zell-LGL-Leukämie · NK-Zell-Leukämie großer granulärer Lymphozyten · NK-Zell-Lymphom, aggressives |
| es | Leucemia agresiva de células NK | ANKCL · Leucemia LGL de células NK · Leucemia linfocítica granular de células NK grandes · Linfoma agresivo de células NK |
| it | Leucemia aggressiva a cellule NK | ANKCL · Leucemia a LGL NK · Leucemia a grandi linfociti granulari NK · Linfoma aggressivo a cellule NK |
| nl | Agressieve NK-celleukemie | ANKCL · Agressief NK-cellymfoom · NK-cel LGL-leukemie · NK-cel groot granulair lymfocytleukemie |
| pt | Leucemia das células NK, agressiva | |
| pl | Agresywna białaczka z komórek NK | Agresywny chłoniak z komórek NK · ANKCL · Białaczka limfocytarna z dużych ziarnistych komórek NK · NK-cell LGL leukemia |
| cs | Agresivní NK-buněčná leukémie | ANKCL · Agresivní NK-buněčný lymfom · NK-buněčná LGL leukémie · NK-velkobuněčná granulární lymfocytární leukémie |
| uk | Агресивна NK-клітинна лейкемія | Агресивна NK-клітинна лімфома · ANKCL · NK-клітинна велика гранулярна лімфоцитарна лейкемія · NK-клітинна лейкемія LGL |
| zh | 侵袭性NK细胞白血病 | NK细胞大颗粒淋巴细胞白血病 |
| tr | Agresif NK hücreli lösemi | ANKCL · NK hücreli LGL lösemi · Agresif NK hücreli lenfoma · NK hücreli büyük granüler lenfosit lösemi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C94.7 | NTBT | inclusion | validated |
| ICD-11 | 2A90.3 | E | specific | validated |
| MONDO | 0019470 | E | validated | |
| UMLS | C1292777 | E | validated | |
| GARD | 10493 | E | validated | |
| MedDRA | 10028811 | 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:512034Large granular lymphocyte leukemia
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
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Europe | 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/86873
- This record as JSON: /data/d/86873.json