Treatment of Macrophage Activation Syndrome (MAS) With Anakinra

Who can take part

• two or more Laboratory criteria: 1. Decreased platelet count (≤262 ×10 9/L) 2. Elevated levels of aspartate aminotransferase (>59 U/L) 3. Decreased white blood cell count (≤4.0 × 109/L) 4. Hypofibrinogenemia (≤2.5 g/L) or, three or more combined clinical/laboratory criteria:
• Decreased platelet count (≤262 × 109/L)
• Elevated levels of aspartate aminotransferase (>59 U/L)
• Decreased white blood cell count (≤4.0 × 109/L)
• Hypofibrinogenemia (≤2.5 g/L)
• Central nervous system dysfunction (irritability, disorientation, lethargy, seizures, coma)
• Hemorrhages (purpura, easy bruising, mucosal bleeding)
• Hepatomegaly (≥3 cm below the costal margin or confirmed by imaging); OR; 2] No previous diagnosis of sJIA and serum ferritin > 2,000 ng/ml and 3 out of the following:
• Bicytopenia with two of the following:; Absolute Neutrophil Count < 1,000,; Platelets < 100, 000/mm3,; Hemoglobin < 9 mg/dl
• Fasting triglyceride >265 mg/dL
• Splenomegaly
• ALT OR AST > 120 IU/L (or > 2x upper limit of normal)
• Fever with temp ≥ 101° F
• Fibrinogen < 1.5 g/L (150 mg/dl) or INR > 1.5 or d-dimer > 500 ng/ml; -

Who cannot take part

• Previous treatment for the current MAS episode with corticosteroids, anakinra, tocilizumab, anti-TNF therapy or cyclosporine
• Evidence of malignancy
• Culture evidence of systemic bacterial infection at the time of screening
• Family history of familial HLH
• Evidence of any of the following; Creatinine at the time of screening > 2X ULN or > twofold increase from patient's baseline creatinine within past 3 months (if known); Albumin < 1.5 at the time of screening; Mechanical ventilation at the time of screening; Hypotension requiring use of pressors at the time of screening
• <1 year of age
• Known EBV viremia by PCR at time of screening (positive serologies are not an exclusion; results of EBV testing will not be necessary for enrollment, but may be ordered as part of the standard of care assessment to guide future management as results become available)