loading...| Preferred Name |
Aggressive systemic mastocytosis |
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| Synonyms |
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| Definitions |
A rare, aggressive form of advanced systemic mastocytosis (advSM) characterized by massive infiltration of mast cells (MC) in different tissues and presence of extracutaneous organ dysfunction, but without evidence of mast cell leukemia or another hematologic neoplasm. |
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| ID |
http://www.orpha.net/ORDO/Orphanet_98850 |
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| definition |
A rare, aggressive form of advanced systemic mastocytosis (advSM) characterized by massive infiltration of mast cells (MC) in different tissues and presence of extracutaneous organ dysfunction, but without evidence of mast cell leukemia or another hematologic neoplasm. |
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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=98850 |
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| has_age_of_onset |
Adult Antenatal Elderly Neonatal |
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| has_inheritance |
Not applicable |
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| hasDbXref |
MedDRA:10056453 UMLS:C1112486 ICD-10:C96.2 ICD-11:2A21.0Y ICD-11:XH10N1 |
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| label |
Aggressive systemic mastocytosis |
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| notation |
ORPHA:98850 |
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| part_of | ||
| prefixIRI |
ORDO:Orphanet_98850 |
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| prefLabel |
Aggressive systemic mastocytosis |
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| present_in |
Europe AND has_point_prevalence_range : 1-9 / 1 000 000 |
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| treeView | ||
| subClassOf |