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Table 3 HLH/MAS Evidence-Based Guideline Treatment Algorithm

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

Illness Severity Serious Infection Potential Medications Dosinga
Moderate Yes Anakinra 2–4 mg/kg/dose (max 100 mg) IV/SQ Q12-24 h
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
  1. HLH hemophagocyticlymphohistiocytosis, MAS macrophage activation syndrome, IVIG intravenous immunoglobulin, methylpred methylprednisolone
  2. 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