loading...| Preferred Name |
Thrombotic thrombocytopenic purpura |
|
| Synonyms |
Moschcowitz disease |
|
| 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. |
|
| ID |
http://www.orpha.net/ORDO/Orphanet_54057 |
|
| 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. |
|
| definition_citation |
Orphanet |
|
| expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=54057 |
|
| has_age_of_onset |
All ages |
|
| has_inheritance |
Multigenic/multifactorial Autosomal recessive |
|
| hasDbXref |
MedDRA:10043648 UMLS:C0034155 ICD-10:M31.1 MeSH:D011697 ICD-11:3B64.14 OMIM:274150 |
|
| label |
Thrombotic thrombocytopenic purpura |
|
| notation |
ORPHA:54057 |
|
| part_of |
http://www.orpha.net/ORDO/Orphanet_506213 |
|
| prefixIRI |
ORDO:Orphanet_54057 |
|
| 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 |
|
| subClassOf |