HLH and MAS diagnostic and classification criteria

	Clinical criteria
	2004 HLH criteria^[1]	2004 MAS criteria^[2]	2016 MAS classification criteria^[3]
Diagnostic requirements	Requires >3 criteria	Requires 2 laboratory or >3 clinical and/or laboratory criteria in addition to fever in a patient with systemic JIA	Requires ferritin criterion and any other 2 criteria in addition to fever in a patient with systemic JIA
Fever	>38.5°C	Present	Present
Organomegaly	Splenomegaly	Hepatomegaly	N/A
Cytopenia	2 of 3 cell lines must be affected	Platelet count ≤262,000/microL OR WBC ≤4000 cells/microL	Platelet count <181,000/microL
Miscellaneous laboratory findings	Triglycerides >265 mg/dL OR Fibrinogen <1.5 g/L	AST >59 units/L	AST >48 units/L, Fibrinogen <360 mg/dL, OR Triglycerides >156 mg/dL
Ferritin	>500 ng/mL	N/A	>684 ng/mL
Other clinical features	N/A	CNS dysfunction Hemorrhages	N/A

HLA: hereditary lymphohistiocytic hemophagocytosis; MAS: macrophage activation syndrome; JIA: juvenile idiopathic arthritis; N/A: not applicable; WBC: white blood cell; AST: aspartate aminotransferase; CNS: central nervous system.

References:

Henter JI, Horne A, Arico M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007; 48:124.
Ravelli A, Magni-Manzoni S, Pistorio A, et al. Preliminary diagnostic guidelines for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. J Pediatr 2005; 146:598.
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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HLH and MAS diagnostic and classification criteria