loading...| Preferred Name |
Hemoglobin H disease |
|
| Synonyms |
HbH disease |
|
| Definitions |
An intermediate form of alpha-thalassemia characterized by increased hemolysis and mild to severe anemia with marked microcytosis and hypochromia. Hemoglobin H disease (HbH) disease belongs to the group of nontransfusion-dependent thalassemia. |
|
| ID |
http://www.orpha.net/ORDO/Orphanet_93616 |
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| alternative_term |
HbH disease Alpha-thalassemia intermedia |
|
| definition |
An intermediate form of alpha-thalassemia characterized by increased hemolysis and mild to severe anemia with marked microcytosis and hypochromia. Hemoglobin H disease (HbH) disease belongs to the group of nontransfusion-dependent thalassemia. |
|
| definition_citation |
Orphanet |
|
| expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=93616 |
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| has_age_of_onset |
All ages |
|
| has_inheritance |
Autosomal recessive |
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| hasDbXref |
ICD-10:D56.0 UMLS:C3161174 ICD-11:3A50.02 OMIM:613978 MedDRA:10063435 |
|
| label |
Hemoglobin H disease |
|
| notation |
ORPHA:93616 Clinical subtype |
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| part_of | ||
| prefixIRI |
ORDO:Orphanet_93616 |
|
| prefLabel |
Hemoglobin H disease |
|
| present_in |
United States AND has_birth_prevalence_average_value : 8.7 AND has_birth_prevalence_range : 1-9 / 100 000 United States AND has_point_prevalence_average_value : 0.1 AND has_point_prevalence_range : 1-9 / 1 000 000 South East Asia AND has_annual_incidence_average_value : 1200.0 AND has_annual_incidence_range : >1 / 1000 |
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| treeView | ||
| subClassOf |