Hemophagocytic Lymphohistiocytosis
GPTKB entity
Statements (49)
| Predicate | Object |
|---|---|
| gptkbp:instanceOf |
gptkb:disease
|
| gptkbp:abbreviation |
gptkb:HLH
|
| gptkbp:affects |
immune system
hematopoietic system |
| gptkbp:alternativeName |
gptkb:familial_hemophagocytic_lymphohistiocytosis
hemophagocytic syndrome |
| gptkbp:associatedWith |
gptkb:PRF1
STX11 STXBP2 UNC13D |
| gptkbp:causedBy |
infection
genetic disorder autoimmune disease malignancy |
| gptkbp:complication |
death
multi-organ failure |
| gptkbp:firstDescribed |
1952
|
| gptkbp:hasDiagnosticMethod |
bone marrow biopsy
triglyceride level ferritin level fibrinogen level soluble IL-2 receptor |
| gptkbp:hasOrphanetID |
ORPHA:399
|
| gptkbp:hasRelatedCondition |
gptkb:macrophage_activation_syndrome
|
| https://www.w3.org/2000/01/rdf-schema#label |
Hemophagocytic Lymphohistiocytosis
|
| gptkbp:ICD-10_code |
D76.1
|
| gptkbp:inheritance |
autosomal recessive
|
| gptkbp:mortalityRate |
high
|
| gptkbp:OMIM |
267700
|
| gptkbp:onset |
childhood
adulthood |
| gptkbp:pathology |
gptkb:cytokine_storm
uncontrolled immune activation |
| gptkbp:prevalence |
rare
|
| gptkbp:prognosis |
poor without treatment
|
| gptkbp:subspecies |
primary HLH
secondary HLH |
| gptkbp:symptom |
fever
enlarged liver enlarged spleen rash lymphadenopathy neurological symptoms pancytopenia |
| gptkbp:treatment |
chemotherapy
immunosuppressive therapy hematopoietic stem cell transplantation |
| gptkbp:bfsParent |
gptkb:HLH
|
| gptkbp:bfsLayer |
8
|