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Comparison classification and diagnostic criteria for MAS and HLH in sJIA

Comparison classification and diagnostic criteria for MAS and HLH in sJIA
  Clinical criteria
2016 MAS classification criteria[1] 2004 HLH criteria*[2]
Diagnostic requirements
  • Requires fever, elevated ferritin, and any other 2 criteria in a patient with sJIA
  • Requires >3 criteria
Fever
  • Present (no specific temperature requirement)
  • >38.5°C
Organomegaly
  • N/A
  • Splenomegaly
Cytopenia
  • Platelet count <181,000/microL
At least 2 of the following:
  • Absolute neutrophil count <1000/microL
  • Hemoglobin <9 g/dL (for infants <4 weeks, hemoglobin <10 g/dL)
  • Platelets <100,000/microL
Miscellaneous laboratory findings
  • AST >48 units/L
  • or
  • Fibrinogen <360 mg/dL
  • or
  • Triglycerides >156 mg/dL
  • Triglycerides >265 mg/dL
  • or
  • Fibrinogen <1.5 g/L
Ferritin
  • >684 ng/mL
  • >500 ng/mL
To be used with UpToDate content on MAS and sJIA.

AST: aspartate aminotransferase; HLA: hereditary lymphohistiocytic hemophagocytosis; MAS: macrophage activation syndrome; N/A: not applicable; sJIA: systemic juvenile idiopathic arthritis; WBC: white blood cell.

* The 2004 HLH diagnostic criteria often cannot be applied to MAS in patients with sJIA because they are too stringent to identify early signs of MAS.
References:
  1. Henter JI, Horne A, Arico M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007; 48:124.
  2. Ravelli A, Minoia F, Davi S, et al. 2016 Classification criteria for macrophage activation syndrome complicating juvenile idiopathic arthritis: A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. Arthritis Rheumatol 2016; 68:566.
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