Acute myeloblastic leukemia without maturation
Synonyms: AML M1 · Acute myeloblastic leukemia M1
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare, acute myeloid leukemia characterized by no significant myeloid maturation and more than 90% blast cells in the non-erythroid population. Various degrees of anemia, thrombocytopenia, or pancytopenia are present. Frequent clinical manifestations include fatigue, fever, bleeding disorders, and organomegaly, especially hepatosplenomegaly.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Acute myeloblastic leukemia without maturation | AML M1 · Acute myeloblastic leukemia M1 |
| fr | Leucémie myéloblastique aiguë sans maturation | AML M1 · LAM M1 · Leucémie myéloblastique aiguë type 1 |
| de | Leukämie, akute myeloische, ohne Ausreifung | AML M1 · Akute myeloische Leukämie M1 |
| es | Leucemia mieloblástica aguda sin maduración | AML M1 · Leucemia mielobástica aguda M1 |
| it | Leucemia mieloblastica acuta senza maturazione | AML M1 · Leucemia mieloblastica acuta M1 · Leucemia mieloide acuta associata alla traslocazione t (8;21) (q22;q22) |
| nl | Acute myeloblastische leukemie zonder maturatie | AML M1 · Acute myeloblastische leukemie M1 |
| pt | Leucemia mieloblástica, aguda, sem maturação | |
| pl | Ostra białaczka mieloblastyczna bez cech dojrzewania | AML M1 · Ostra białaczka mieloblastyczna M1 |
| cs | Akutní myeloblastická leukémie bez vyzrávání | AML M1 · Akutní myeloblastická leukémie M1 |
| uk | Гостра мієлобластна лейкемія без дозрівання | Гострий мієлобластний лейкоз M1 · ГМЛ M1 |
| zh | 急性粒细胞白血病未分化型 | M2型急性髓性白血病 |
| tr | Olgunlaşmamış akut miyeloblastik lösemi | AML M1 · Akut miyeloblastik lösemi M1 |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C92.0 | NTBT | attributed | validated |
| ICD-11 | 2A60.31 | NTBT | index | validated |
| MONDO | 0005224 | E | validated | |
| UMLS | C0026998 | E | validated | |
| GARD | 526 | 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:167714Unclassified acute myeloid leukemia
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
No children.
Genes
2 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| FLT3 | Disease-causing somatic mutation(s) in | Assessed | HGNC:3765 | ENSG00000122025 | 136351 | 13q12.2 | fms related receptor tyrosine kinase 3 | 22338050[PMID] |
| NPM1 | Disease-causing somatic mutation(s) in | Not yet assessed | HGNC:7910 | ENSG00000181163 | 164040 | 5q35.1 | nucleophosmin 1 | 22338050[PMID] |
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.01 / 100 000 | Europe | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[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/98833
- This record as JSON: /data/d/98833.json