HScore for Reactive Hemophagocytic Syndrome

HScore Calculator
Temperature
Organomegaly
Cytopenia (Number of Lineages)
Ferritin (ng/mL)
Triglycerides (mmol/L)
Fibrinogen (g/L)
AST (U/L)
Hemophagocytosis on Bone Marrow Aspirate
Known Immunosuppression (HIV or Chronic Immunosuppressive Therapy)
HScore: –
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Diagnoses reactive hemophagocytic syndrome.

Why Use

HPS and HLH have a high fatality rate if not rapidly identified and treated. This score utilizes a mix of clinical and laboratory findings to help predict this rare diagnosis.

When to Use

In adult patients in whom hemophagocytic syndromes (HPS), including hemophagocytic lymphohistiocytosis (HLH), are suspected. Further validation is needed in children; it is not recommended for use in this population.

Formula

The HScore is calculated by adding the selected points for each variable: Variable Points Known underlying immunosuppression* No 0 Yes 18 Temperature, °F (°C) <101.1 (<38.4) 0 101.1–102.9 (38.4-39.4) 33 >102.9 (>39.4) 49 Organomegaly No 0 Hepatomegaly or splenomegaly 23 Hepatomegaly and splenomegaly 38 Number of cytopenias** 1 lineage 0 2 lineages 24 3 lineages 34 Ferritin, ng/mL (μg/L) <2,000 0 2,000–6,000 35 >6,000 50 Triglycerides, mg/dL (mmol/L) <132.7 (<1.5) 0 132.7-354 (1.5–4) 44 >354 (>4) 64 Fibrinogen, mg/dL (g/L) >250 (>2.5) 0 ≤250 (≤2.5) 30 AST , U/L <30 0 ≥30 19 Hemophagocytosis features on bone marrow aspirate No 0 Yes 35 *HIV positive or receiving long‐term immunosuppressive therapy (i.e., glucocorticoids, cyclosporine, azathioprine). **Defined as hemoglobin ≤9.2 g/dL (≤5.71 mmol/L) and/or WBC ≤5,000/mm³ and/or platelets ≤110,000/mm³.

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.

More Information

Interpretation: HScore Probability of hemophagocytic syndrome ≤90 <1% 91-100 ~1% 101-110 1-3% 111-120 3-5% 121-130 5-9% 131-140 9-16% 141-150 16-25% 151-160 25-40% 161-170 40-54% 171-180 54-70% 181-190 70-80% 191-200 80-88% 201-210 88-93% 211-220 93-96% 221-230 96-98% 231-240 98-99% ≥241 >99% Note: the best cutoff value for the HScore was 169, corresponding to a sensitivity of 93%, specificity of 86%, and accurate classification of 90% of patients.

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