macrophage activation syndrome
MONDO:0015545Mondo
Findings
No curated finding names macrophage activation syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
A complication of rheumatic disease that is caused by excessive activation and uncontrolled proliferation of T lymphocytes and well-differentiated macrophages. It is characterized by fever, pancytopenia, liver insufficiency, coagulopathy and neurologic symptoms.
Definition from the Mondo Disease Ontology (MONDO:0015545), read 2026-09-29. CC BY 4.0.
Features
29 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, annotations 2026-09-02.
- Abnormal circulating interleukin concentrationHPOHP:0011117
- Very frequent (80% to 99% of cases)
- Abnormal total natural killer cell countHPOHP:0040089
- Very frequent (80% to 99% of cases)
- Abnormality of tumor necrosis factor secretionHPOHP:0011118
- Very frequent (80% to 99% of cases)
- AnemiaHPOHP:0001903
- Very frequent (80% to 99% of cases)
- AutoimmunityHPOHP:0002960
- Very frequent (80% to 99% of cases)
- Decreased liver functionHPOHP:0001410
- Very frequent (80% to 99% of cases)
- Elevated circulating alanine aminotransferase concentrationHPOHP:0031964
- Very frequent (80% to 99% of cases)
- Elevated erythrocyte sedimentation rateHPOHP:0003565
- Very frequent (80% to 99% of cases)
- FeverHPOHP:0001945
- Very frequent (80% to 99% of cases)
- HemophagocytosisHPOHP:0012156
- Very frequent (80% to 99% of cases)
- HypoalbuminemiaHPOHP:0003073
- Very frequent (80% to 99% of cases)
- Increased circulating ferritin concentrationHPOHP:0003281
- Very frequent (80% to 99% of cases)
Show the remaining 17
- Increased circulating interferon-gamma concentrationHPOHP:0030356
- Very frequent (80% to 99% of cases)
- Increased circulating interleukin 6 concentrationHPOHP:0030783
- Very frequent (80% to 99% of cases)
- Increased circulating lactate dehydrogenase concentrationHPOHP:0025435
- Very frequent (80% to 99% of cases)
- Increased inflammatory responseHPOHP:0012649
- Very frequent (80% to 99% of cases)
- ThrombocytopeniaHPOHP:0001873
- Very frequent (80% to 99% of cases)
- Decreased total neutrophil countHPOHP:0001875
Where it sits
Other names
2 names
Resolves to: macrophage activation syndrome
- Also called
- MASreactive hemophagocytic lymphohistiocytosis