Acute myeloid leukemia and myelodysplastic syndromes related to radiation
Synonyms: AML and myelodysplastic syndromes related to radiation
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult, Elderly
- Status
- active
A subgroup of therapy-related myeloid neoplasms (t-MN), associated with treatment of an unrelated neoplastic disease with radiation. The neoplastic cells typically harbor unbalanced aberrations of chromosomes 5 and 7 (monosomy 5/del(5q) and monosomy 7/del(7q)) or a complex karyotype. Patients frequently present with multilineage dysplasia and cytopenias 5-10 years after exposure.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Acute myeloid leukemia and myelodysplastic syndromes related to radiation | AML and myelodysplastic syndromes related to radiation |
| fr | Leucémie aiguë myéloïde et syndromes myélodysplasiques liés aux radiations | |
| de | Akute myeloische Leukämie und Myelodysplastische Syndrome durch Strahlung | AML und MDS durch Strahlung |
| es | Leucemia mieloide aguda y síndromes mielodisplásicos relacionados con la radiación | LMA y síndromes mielodisplásicos asociados a radiación |
| it | Leucemia mieloide acuta e sindromi mielodisplastiche correlate alle radiazioni | AML e sindromi mielodisplastiche, associate a radiazioni |
| nl | Acute myeloïde leukemie en myelodysplastische syndromen gerelateerd aan bestraling | AML en myelodysplastische syndromen gerelateerd aan bestraling |
| pt | Leucemia mielóide aguda e síndromes mielodisplásicos relacionados com radiação | |
| pl | Ostra białaczka szpikowa i zespoły mielodysplastyczne związane z promieniowaniem | AML i zespoły mielodysplastyczne związane z promieniowaniem |
| cs | Akutní myeloidní leukémie a myelodysplastické syndromy související s radiací | AML a myelodysplastický syndrom související s radiací |
| uk | Гостра мієлоїдна лейкемія та мієлодиспластичні синдроми, повязані з опроміненням | ГМЛ та мієлодиспластичні синдроми, повязані з опроміненням |
| zh | 急性髓系白血病与辐射相关骨髓增生异常综合征 | AML与辐射相关骨髓增生异常综合征 |
| tr | Akut miyeloid lösemi ve radyasyona bağlı miyelodisplastik sendromlar | Radyasyona bağlı AML ve miyelodisplastik sendromlar |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D46.7 | NTBT | attributed | validated |
| ICD-11 | 2A60.20 | NTBT | attributed | validated |
| MONDO | 0015608 | E | validated | |
| UMLS | C4707660 | 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 (2):
- ORPHA:521132Radiation-induced disorder
rare disorders due to toxic effects - ORPHA:86846Therapy related acute myeloid leukemia and myelodysplastic syndrome
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 / 1 000 000 | 0.3 / 100 000 | Worldwide | Value and class | Validated | 22585703[PMID]_39256810[PMID]_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/164726
- This record as JSON: /data/d/164726.json