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Refractory anemia with excess blasts
Disease definition
Refractory anemia with excess blasts (RAEB) is a frequent severe subtype of myelodysplastic syndrome (MDS; see this term) characterized by cytopenias with unilineage or multilineage dysplasia and 5% to 19% blasts in bone marrow or blood.
ORPHA:86839
Classification level: Disorder- Synonym(s):
- RAEB
- Prevalence: Unknown
- Inheritance: Not applicable
- Age of onset: Adult
- ICD-10: D46.2
- ICD-11: 2A35
- OMIM: -
- UMLS: C0002894
- MeSH: D000754
- GARD: -
- MedDRA: 10038270
Summary
Epidemiology
Exact prevalence is unknown but RAEB accounts for about 40% of patients with MDS which has a prevalence estimated to be 1/25,000 to 1/33,000. The disease tends to occur in older adults with a male predominance.
Clinical description
Two types of RAEB are recognized: type 1 and type 2 (RAEB-1 and RAEB-2; see these terms) which are differentiated based on the percentage of blasts in bone marrow or blood. Patients usually present with symptoms related to one or more cytopenias. Survival is lower in patients with RAEB-2 and there is a higher rate of transformation into acute myeloid leukemia in these patients. Most RAEB patients die from neutropenia-, thrombocytopenia- and anemia-related complications.
Etiology
The etiology is not known but is thought to involve inherited susceptibility or hematopoietic stem cell damage.
A summary on this disease is available in Español (2015) Italiano (2015) Nederlands (2015)
Detailed information
Guidelines
- Clinical practice guidelines
- Français (2008) - PNDS
- English (2013) - Blood
- English (2015) - Blood
- English (2017) - Blood
Disease review articles
- Review article
- Deutsch (2021) - Onkopedia
- English (2021) - Onkopedia
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produced/endorsed by FSMR(s)Additional information