Calm in the midst of cytokine storm: a collaborative approach to the diagnosis and treatment of hemophagocytic lymphohistiocytosis and macrophage activation syndrome

Table 3 HLH/MAS Evidence-Based Guideline Treatment Algorithm

Illness Severity	Serious Infection	Potential Medications	Dosing^a
Moderate	Yes	Anakinra	2–4 mg/kg/dose (max 100 mg) IV/SQ Q12-24 h
	Yes	IVIG	1–2 g/kg/dose IV
	No	Anakinra	2–4 mg/kg/dose (max 100 mg) IV/SQ Q12-24 h
		Methylpred	1 mg/kg/dose IV Q12hrs OR 30 mg/kg/dose (max 1 g) IV Q24 hrs × 3 doses
		Cyclosporine (Neoral®)	3–7 mg/kg/day PO Q12hrs
		Tacrolimus	0.1 mg/kg/day PO Q12 hrs
		IVIG	1–2 g/kg/dose IV
Critical	N/A	Anakinra	2–4 mg/kg/dose IV/SQ Q6-24 h (can go higher)
		Methylpred	30 mg/kg/dose (max 1 g) IV Q24 hrs × 3 doses➔1 mg/kg/dose IV Q12hrs
		Cyclosporine	3–7 mg/kg/day PO or 3–5 mg/kg/day IV Q12hrs (enteral preferred)
		Tacrolimus	0.1 mg/kg/day PO or 0.01–0.05 mg/kg/day IV (enteral preferred)
		IVIG	1–2 g/kg/dose IV

HLH hemophagocyticlymphohistiocytosis, MAS macrophage activation syndrome, IVIG intravenous immunoglobulin, methylpred methylprednisolone
^aThe medication dosing contained within these guidelines is provided as a reference only. Please refer to institutional formulary or ordering guidelines when placing orders for the clinical care of patients

ISSN: 1546-0096