loading...| Preferred Name | Familial hemophagocytic lymphohistiocytosis | |
| Synonyms |
Familial HLH |
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| Definitions |
Familial Hemophagocytic lymphohistiocytosis (FHL) is a rare primary immunodeficiency characterized by a macrophage activation syndrome (see this term) with an onset usually occurring within a few months or less common several years after birth. |
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| ID |
http://www.orpha.net/ORDO/Orphanet_540 |
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| database_cross_reference |
MedDRA:10070916 OMIM:603552 OMIM:603553 ICD10:D76.1 OMIM:613101 OMIM:267700 OMIM:608898 UMLS:C0272199 MedDRA:10070904 |
|
| definition |
Familial Hemophagocytic lymphohistiocytosis (FHL) is a rare primary immunodeficiency characterized by a macrophage activation syndrome (see this term) with an onset usually occurring within a few months or less common several years after birth. |
|
| definition_citation |
orphanet |
|
| has_exact_synonym |
Familial HLH |
|
| label |
Familial hemophagocytic lymphohistiocytosis |
|
| preferred label |
Familial hemophagocytic lymphohistiocytosis |
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| prefLabel |
Familial hemophagocytic lymphohistiocytosis |
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| subClassOf |