MDCalc

HScore for Reactive Hemophagocytic Syndrome

Diagnoses reactive hemophagocytic syndrome.

Known underlying immunosuppression

HIV positive or receiving long‐term immunosuppressive therapy (i.e., glucocorticoids, cycloSPORINE, azaTHIOprine)

Temperature, °F (°C)

Organomegaly

Number of cytopenias

Defined as hemoglobin ≤9.2 g/dL (≤5.71 mmol/L) and/or WBC ≤5,000/mm³ and/or platelets ≤110,000/mm³

Ferritin, ng/mL (or μg/L)

Triglycerides, mg/dL (mmol/L)

Fibrinogen, mg/dL (g/L)

AST, U/L

Hemophagocytosis features on bone marrow aspirate

Result:

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Management
  • Management of HPS and HLH includes identifying and treating the disease trigger and following the clinical protocols set out by the Histiocyte Society. Two commonly referenced protocols include HLH-94 and HLH-2004.
  • Portions of the HLH-2004 protocol are still being studied, and HLH-94 is the more frequently utilized protocol. 
  • Prompt consultation with a specialist in HLH is recommended.
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