Refractory anemia with excess blasts in transformation
Synonyms: RAEB-t
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adolescent, Adult, Elderly
- Status
- active
A rare hematologic disease characterized by the presence of 20-29% blasts in the bone marrow, presence of 5-29% blasts in the peripheral blood, and/or presence of Auer rods. Patients show relatively stable peripheral blood counts for weeks or months, with specific cytogenetic and molecular genetic characteristics constituting important prognostic factors.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Refractory anemia with excess blasts in transformation | RAEB-t |
| fr | Anémie réfractaire avec excès de blastes en transformation | = en |
| de | Refraktäre Anämie mit Vermehrung von Blasten in Transformation | = en |
| es | Anemia refractaria con exceso de blastos en transformación | AREB-t |
| it | Anemia refrattaria con eccesso di blasti in trasformazione | = en |
| nl | Refractaire anemie met exces aan blasten in transformatie | = en |
| pt | Anemia refratária com excesso de blastos em transformação | |
| pl | Niedokrwistość oporna na leczenie z transformacją białaczkową | = en |
| cs | Refrakterní anémie s nadbytkem blastů | RAEB-T |
| uk | Рефрактерна анемія з надлишком бластів у стадії трансформації | = en |
| zh | 未成熟细胞过多的难治性贫血 | = en |
| tr | Transformasyonda artmış blast içeren refrakter anemi | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D46.2 | NTBT | inclusion | validated |
| ICD-11 | 2A34 | NTBT | index | validated |
| MONDO | 0015692 | E | validated | |
| UMLS | C0280028 | E | validated | |
| MedDRA | 10038271 | 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:52688Myelodysplastic 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 / 1 000 000 | 0.04 / 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/168960
- This record as JSON: /data/d/168960.json