HLH-like complication of rheumatic disease, especially systemic juvenile idiopathic arthritis.
- Specialty
- Hematology
- Subspecialty
- Hematology / Histiocytic disorders
- Sex & age distribution
- Male: affected; Female: affected; Children: yes
- Gene(s) involved
- PRF1, STXBP2, UNC13D
- Alternative names & eponyms
- MAS
Etiology & mechanism
Cytokine storm with macrophage activation.
Gene(s) involved (3)
Symptoms & signs (6)
In plain terms, someone with Macrophage activation syndrome may experience the following — click any to explore related syndromes:
Same gene(s)
Other syndromes that involve one of this syndrome's genes.
Overlapping symptoms
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Shares symptoms with
2 shared
Castleman syndrome
cytopenias, fever
2 shared
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cytopenias, fever
1 shared
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fever
1 shared
Acute chest syndrome
fever
1 shared
Acute fatty liver of pregnancy syndrome
coagulopathy
1 shared
Antisynthetase syndrome
fever
1 shared
Behçet-like haploinsufficiency of A20 syndrome
fever
1 shared
Blue rubber bleb nevus syndrome
coagulopathy
1 shared
Boerhaave syndrome
fever
1 shared
Cryopyrin-associated periodic syndrome
fever
Shares genes with
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