Preferred Name |
Paroxysmal nocturnal hemoglobinuria |
|
Synonyms |
PNH |
|
Definitions |
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disorder characterized by corpuscular hemolytic anemia, bone marrow failure and frequent thrombotic events. |
|
ID |
http://www.orpha.net/ORDO/Orphanet_447 |
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alternative_term |
PNH Marchiafava-Micheli disease |
|
definition |
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disorder characterized by corpuscular hemolytic anemia, bone marrow failure and frequent thrombotic events. |
|
definition_citation |
Orphanet |
|
expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=447 |
|
has_age_of_onset |
All ages |
|
has_inheritance |
Not applicable |
|
hasDbXref |
MeSH:D006457 OMIM:300818 MedDRA:10034042 OMIM:615399 UMLS:C0024790 ICD-10:D59.5 ICD-11:3A21.0 |
|
label |
Paroxysmal nocturnal hemoglobinuria |
|
notation |
ORPHA:447 |
|
part_of |
http://www.orpha.net/ORDO/Orphanet_164823 http://www.orpha.net/ORDO/Orphanet_182047 |
|
prefixIRI |
ORDO:Orphanet_447 |
|
prefLabel |
Paroxysmal nocturnal hemoglobinuria |
|
present_in |
Europe AND has_point_prevalence_average_value : 2.0 AND has_point_prevalence_range : 1-9 / 100 000 United Kingdom AND has_annual_incidence_average_value : 0.35 AND has_annual_incidence_range : 1-9 / 1 000 000 United Kingdom AND has_point_prevalence_average_value : 3.81 AND has_point_prevalence_range : 1-9 / 100 000 |
|
treeView |
http://www.orpha.net/ORDO/Orphanet_164823 http://www.orpha.net/ORDO/Orphanet_182047 |
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subClassOf |