Acute myeloid leukemia with t(9;22)(q34.1;q11.2)
Synonyms: AML with BCR-ABL1 · AML with t(9;22)(q34.1;q11.2)
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood, Elderly, Infancy, Neonatal
- Status
- active
A rare acute myeloid leukemia (AML) with recurrent genetic anomaly characterized by the presence of bone marrow and peripheral blood myeloblasts with features ranging from those of minimal differentiation to granulocytic maturation, demonstrating t(9;22)(q34.1;q11.2) or molecular genetic evidence of BCR-ABL1 fusion. Evidence of chronic myeloid leukemia (CML) is absent. Patients most commonly present with leukocytosis with blast predominance and variable anemia and thrombocytopenia. Splenomegaly is less frequent and peripheral blood basophilia lower than in patients with myeloid blast transformation of CML. The disease occurs primarily in adults, and response to traditional AML therapy or tyrosine kinase inhibitor therapy alone is typically poor.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Acute myeloid leukemia with t(9;22)(q34.1;q11.2) | AML with BCR-ABL1 · AML with t(9;22)(q34.1;q11.2) |
| fr | Leucémie myéloïde aiguë associée à t(9;22)(q34.1;q11.2) | AML avec BCR-ABL1 · LAM avec BCR-ABL1 · LAM avec t(9;22)(q34.1;q11.2) |
| de | Akute myeloblastische Leukämie mit t(9;22)(q34.1;q11.2) | |
| es | Leucemia mieloide aguda con t(9;22)(q34.1;q11.2) | LMA con BCR-ABL1 · LMA con t(9;22)(q34.1;q11.2) |
| it | Leucemia mieloide acuta con t(9;22)(q34.1;q11.2) | LMA con BCR-ABL1 · LMA con t(9;22)(q34.1;q11.2) |
| nl | Acute myeloïde leukemie met t(9;22)(q34.1;q11.2) | AML met BCR-ABL1 · AML met t(9;22)(q34.1;q11.2) |
| pt | Leucemia mielóide aguda com t(9;22)(q34.1;q11.2) | |
| pl | Ostra białaczka szpikowa z t(9;22)(q34.1;q11.2) | = en |
| cs | Akutní myeloidní leukémie s t(9;22)(q34.1;q11.2) | = en |
| uk | Гострий мієлоїдний лейкоз з t(9;22)(q34.1;q11.2) | AML з BCR-ABL1 · AML з t(9;22)(q34.1;q11.2) |
| zh | Acute myeloid leukemia with t(9;22)(q34.1;q11.2) | = en |
| tr | Acute myeloid leukemia with t(9;22)(q34.1;q11.2) | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C92.0 | NTBT | attributed | validated |
| ICD-11 | 2A60.0 | NTBT | attributed | validated |
| MONDO | 0035112 | E | validated | |
| UMLS | C4329268 | 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:98277Acute myeloid leukemia with recurrent genetic anomaly
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 |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 100 000 | 4 / 100 000 | Worldwide | Value and class | Validated | 29340131[PMID]_29090343[PMID]_31101526[PMID] |
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/585867
- This record as JSON: /data/d/585867.json