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Table 3 HScore

From: Macrophage activation syndrome triggered by systemic lupus erythematosus flare: successful treatment with a combination of dexamethasone sodium phosphate, intravenous immunoglobulin, and cyclosporine: a case report

Parameter

No. of points (criteria for scoring)

Known underlying immunosuppression

0 (no) or 18 (yes)

Temperature (°C)

0 (< 38.4), 33 (38.4–39.4), or 49 (> 39.4)

Organomegaly

0 (no), 23 (hepatomegaly or splenomegaly), or 38 (hepatomegaly and splenomegaly)

Number of cytopenias

0 (one lineage), 24 (two lineages), or 34 (three lineages)

Ferritin (ng/mL)

0 (< 2000), 35 (2000–6000), or 50 (> 6000)

Triglyceride (mmol/L)

0 (< 1.5), 44 (1.5–4), or 64 (> 4)

Fibrinogen (g/L)

0 (> 2.5) or 30 (< 2.5)

AST, U/L

0 (< 30) or 19 (> 30)

Hemophagocytosis features on bone marrow

0 (no) or 35 (yes)

  1. AST Aspartate transaminase