Preferred Name | Thrombotic thrombocytopenic purpura | |
Synonyms |
Moschcowitz disease TTP |
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Definitions |
An aggressive and life-threatening form of thrombotic microangiopathy (TMA) characterized by profound peripheral thrombocytopenia, microangiopathic hemolytic anemia (MAHA) and organ failure of variable severity and is comprised of a congenital (cTTP) and acquired, immune-mediated (iTTP) form. |
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ID |
http://www.orpha.net/ORDO/Orphanet_54057 |
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alternative_term |
Moschcowitz disease TTP |
|
definition |
An aggressive and life-threatening form of thrombotic microangiopathy (TMA) characterized by profound peripheral thrombocytopenia, microangiopathic hemolytic anemia (MAHA) and organ failure of variable severity and is comprised of a congenital (cTTP) and acquired, immune-mediated (iTTP) form. |
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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=54057 |
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has_age_of_onset |
All ages |
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has_inheritance |
Multigenic/multifactorial Autosomal recessive |
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hasDbXref |
MedDRA:10043648 UMLS:C0034155 ICD-10:M31.1 MeSH:D011697 ICD-11:3B64.14 OMIM:274150 |
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label |
Thrombotic thrombocytopenic purpura |
|
notation |
ORPHA:54057 |
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part_of |
http://www.orpha.net/ORDO/Orphanet_506213 |
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prefixIRI |
ORDO:Orphanet_54057 |
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prefLabel |
Thrombotic thrombocytopenic purpura |
|
present_in |
Worldwide AND has_annual_incidence_average_value : 0.35 AND has_annual_incidence_range : 1-9 / 1 000 000 Worldwide AND has_point_prevalence_range : Unknown |
|
treeView |
http://www.orpha.net/ORDO/Orphanet_506213 |
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subClassOf |