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Unclassified myelodysplastic syndrome
Disease definition
Unclassified myelodysplastic syndrome (MDS-U) is a subtype of myelodysplastic syndrome (MDS; see this term) with atypical features of uncertain clinical significance.
ORPHA:98827
Classification level: Disorder- Synonym(s): -
- Prevalence: Unknown
- Inheritance: Not applicable
- Age of onset: All ages
- ICD-10: D46.7
- ICD-11: 2A37
- OMIM: -
- UMLS: C5681636
- MeSH: -
- GARD: -
- MedDRA: -
Summary
Epidemiology
Prevalence is unknown but MDS-U appears to account for a very low proportion of MSD cases.
Clinical description
Unclassified MDS covers cases that cannot be allocated to another subtype of MSD after a full diagnostic work-up has been performed including clinical, morphologic, immunophenotypic, or cytogenetic testing. One or more cytopenias are a standard feature of MDS-U but other clinical features are variable. The following cases are considered to fall into this subtype: features of refractory cytopenia with uni- or multi-lineage dysplasia (RCUD, RCMD, see these terms) but with 1% blasts in peripheral blood, unilineage dysplasia with pancytopenia, and persistent cytopenias without morphological features of MDS associated with characteristic cytogenetic abnormalities.
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
- English (2017) - Semin Hematol
- Deutsch (2021) - Onkopedia
- English (2021) - Onkopedia
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produced/endorsed by FSMR(s)Additional information