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Orphanet Rare Disease Ontology
Last uploaded:
July 3, 2024
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| Preferred Name | Systemic mastocytosis | |
| Synonyms |
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| Definitions |
A heterogeneous group of rare, acquired and chronic hematological malignancies related to an abnormal accumulation/proliferation of neoplastic mast cells (MCs) in one or several organs, mainly the bone marrow (BM), associated frequently with skin involvement. |
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| ID |
http://www.orpha.net/ORDO/Orphanet_2467 |
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| definition |
A heterogeneous group of rare, acquired and chronic hematological malignancies related to an abnormal accumulation/proliferation of neoplastic mast cells (MCs) in one or several organs, mainly the bone marrow (BM), associated frequently with skin involvement.
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| definition_citation |
Orphanet
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| expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=en&Expert=2467 |
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| has_age_of_onset |
All ages
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| has_inheritance |
Not applicable
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| hasDbXref |
MeSH:D034721 MedDRA:10042949 ICD-10:C96.2 ICD-11:2A21.0 UMLS:C0221013
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| label |
Systemic mastocytosis
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| notation |
Clinical group ORPHA:2467
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| prefixIRI |
ORDO:Orphanet_2467
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| prefLabel |
Systemic mastocytosis
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| present_in |
Denmark AND has_annual_incidence_average_value : 0.9 AND has_annual_incidence_range : 1-9 / 1 000 000 Worldwide AND has_point_prevalence_range : 1-5 / 10 000 Europe AND has_point_prevalence_average_value : 11.3 AND has_point_prevalence_range : 1-5 / 10 000 Europe AND has_annual_incidence_average_value : 0.6 AND has_annual_incidence_range : 1-9 / 1 000 000 Denmark AND has_point_prevalence_average_value : 9.6 AND has_point_prevalence_range : 1-9 / 100 000 Netherlands AND has_point_prevalence_average_value : 13.0 AND has_point_prevalence_range : 1-5 / 10 000 Italy AND has_annual_incidence_average_value : 0.3 AND has_annual_incidence_range : 1-9 / 1 000 000
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| subClassOf |
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