hemophagocytic syndrome
Findings
No curated finding names hemophagocytic syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Hemophagocytic syndrome (HPS) is a rare immune disease 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), 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).
Definition from the Mondo Disease Ontology (MONDO:0015540), read 2026-09-29. CC BY 4.0.
Features
3 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, hp/releases/2026-09-01.
- HepatosplenomegalyMondoHP:0001433
- LymphadenopathyMondoHP:0002716
- PancytopeniaMondoHP:0001876
Where it sits
Other names
2 names
Resolves to: hemophagocytic syndrome
- Also called
- Hemophagocytic LymphohistiocytosisHLH