Macrophage Activation Syndrome: A Report of Two Cases and a Literature Review

Table 2

Diagnostic criteria for macrophage activation syndrome: Parodi’s preliminary diagnostic guidelines for MAS as a complication of juvenile SLE.

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The diagnosis of MAS requires the simultaneous presence of at least 1 clinical criterion and at least 2 laboratory criteria

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Clinical criteria

(1) Fever (>38°C)

(2) Hepatomegaly (≥3 cm below the costal arch)

(3) Splenomegaly (≥3 cm below the costal arch)

(4) Hemorrhagic manifestations (purpura, easy bruising, or mucosal bleeding)

(5) Central nervous system dysfunction (irritability, disorientation, lethargy, headache, seizures, or coma)

Laboratory criteria

(1) Cytopenia affecting 2 or more cell lineages (white blood cell count ≤ 4.0 × 10⁹/L, hemoglobin ≤ 90 g/L, or platelet count ≤ 150 × 10⁹/L)

(2) Increased aspartate aminotransferase (>40 U/L)

(3) Increased lactate dehydrogenase (>567 U/L)

(4) Hypofibrinogenemia (fibrinogen ≤ 1.5 g/L)

(5) Hypertriglyceridemia (triglycerides > 178 mg/dl)

(6) Hyperferritinemia (ferritin > 500 µg/L)

Histopathologic criterion

Evidence of macrophage hemophagocytosis in the bone marrow aspirate

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Bone marrow aspiration for evidence of macrophage hemophagocytosis may be required only in doubtful cases. Table 2 is copied from Parodi et al. [18].