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Table 2 H-Score for reactive hemophagocytic syndrome in our patient

From: Macrophage activation syndrome, a rare complication of primary Sjögren’s syndrome: a case report

Criteria

Score

Know underlying immunosuppression (that is, HIV positive or receiving long-term immunosuppressive therapy, such as glucocorticoids, cyclosporine, and azathioprine

Yes (+ 18 points)

Temperature (°C)

39.5 °C (+ 49 points)

Organomegaly

No (0)

Number of cytopenia (defined as hemoglobin ≤ 9.2 g/dl and/or WBC ≤ 5000/mm3 and/or platelets ≤ 110,000/mm3)

2 lineages (+ 24 points)

Ferritin (ng/ml)

1778 (0)

Triglyceride (mg/dl)

195 (+ 44)

Fibrinogen (g/l)

2 (30)

AST (U/L)

35 (+ 19 points)

Hemophagocytosis features on bone marrow aspirate

Yes (+ 35 points)

Total

219 points

  1. Probability of hemophagocytic syndrome in our patient is 93–96%. Optimal cut-off 169 points. AST aspartate transferase, WBC white blood cell, HIV human immunodeficiency virus