Acute undifferentiated leukemia
Synonyms: Acute myeloid leukemia, minimal differentiation, FAB M0
- Type
- Disease
- Group
- Disorder
- Status
- active
A rare acute leukemia of ambiguous lineage characterized by clonal proliferation of primitive hematopoietic cells, primarily in the bone marrow and blood, lacking lineage-specific markers and detectable genotypic alterations. The patients present with leukocytosis, anemia, variable platelet count and a variety of nonspecific symptoms related to ineffective hematopoesis (fatigue, bleeding and bruising, recurrent infections, bone pain) and/or extramedullary site involvement (lymphadenopathy, splenomegaly, hepatomegaly).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Acute undifferentiated leukemia | Acute myeloid leukemia, minimal differentiation, FAB M0 |
| fr | Leucémie aiguë non différenciée | Leucémie myéloïde aiguë avec différenciation minimale (FAB M0) |
| de | Leukämie, akute undifferenzierte | Minimal differenzierte akute myeloische Leukämie, FAB M0 |
| es | Leucemia aguda indiferenciada | Leucemia mieloide aguda, diferenciación mínima, FAB M0 |
| it | Leucemia acuta indifferenziata | |
| nl | Acute ongedifferentieerde leukemie | Acute myeloïde leukemie, minimale differentiatie, FAB MO |
| pt | Leucemia, indiferenciada, aguda | |
| pl | Ostra białaczka niezróżnicowana | = en |
| cs | Akutní nediferencovaná leukémie | = en |
| uk | Гострий недиференційований лейкоз | Гостра мієлоїдна лейкемія, мінімальна диференціація, FAB M0 |
| zh | 急性未分化白血病 | 微分化急性髓系白血病FAB M0型 |
| tr | Akut farklılaşmamış lösemi | Akut miyeloid lösemi, minimal farklılaşma, FAB M0 |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 601626 | NTBT | validated | |
| ICD-10 | C95.0 | NTBT | attributed | validated |
| ICD-11 | 2B33.0 | NTBT | attributed | validated |
| MONDO | 0020321 | E | validated | |
| UMLS | C0280141 | E | validated | |
| MedDRA | 10073479 | 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:86851Acute leukemia of ambiguous lineage
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
No children.
Genes
1 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| KMT2A | Part of a fusion gene in | Assessed | HGNC:7132 | ENSG00000118058 | 159555 | 11q23.3 | lysine methyltransferase 2A | 11579461[PMID] |
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/98835
- This record as JSON: /data/d/98835.json