loading...| Preferred Name |
Hemophagocytic syndrome |
| ID |
http://www.orpha.net/ORDO/Orphanet_158032 |
| alternative_term |
Hemophagocytic lymphohistiocytosis HLH |
| definition |
Hemophagocytic syndrome (HPS) is a rare immune disease (see this term) and a potentially life-threatening disorder characterized by cytokine storm and overwhelming inflammation causing fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hyperferritinemia, and hemophagocytosis in bone marrow, liver, spleen or lymph nodes. It can be either primary due to a genetic defect (primary hemophagocytic lymphohistiocytosis ; see this term), or secondary to malignancies, to infections, most commonly with viruses such as Epstein-Barr virus or cytomegalovirus, human immunodeficiency virus, or to autoimmune disorders such as systemic lupus erythematosus or adult-onset Still disease (secondary hemophagocytic lymphohistiocytosis) (see these termes). |
| definition_citation |
Orphanet |
| expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=158032 |
| has_age_of_onset |
Adolescent Childhood Infancy |
| hasDbXref |
ICD-11:4A01.23 UMLS:C0024291 MedDRA:10058125 |
| label |
Hemophagocytic syndrome |
| notation |
Category ORPHA:158032 |
| prefixIRI |
ORDO:Orphanet_158032 |
| prefLabel |
Hemophagocytic syndrome |
| present_in |
Worldwide AND has_point_prevalence_range : Unknown United Kingdom AND has_annual_incidence_average_value : 0.198 AND has_annual_incidence_range : 1-9 / 100 000 |
| subClassOf |