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The HScore

Using the HScore to diagnose hemophagocytic syndromes1

The HScore is a diagnostic tool developed to estimate a patient’s risk of having a hemophagocytic syndrome, such as MAS. It features 9 clinical, biologic, and cytologic parameters that are individually weighted.

Parameter

Number 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 (1 lineage), 24 (2 lineages), or 34 (3 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)

Serum glutamic oxaloacetic transaminase (IU/L)

0 (<30) or 19 (≥30)

Hemophagocytosis features on bone marrow aspirate

0 (no) or 35 (yes)

*Human immunodeficiency virus positive or receiving long-term immunosuppressive therapy (ie, glucocorticoids, cyclosporine, azathioprine).
Defined as a hemoglobin level of ≤9.2 g/dL and/or a leukocyte count of ≤5000/mm3 and/or a PLT count of ≤110,000/mm3.

Probability of a hemophagocytic syndrome, such as MAS,
according to the patient’s HScore

HScore

Probability of hemophagocytic syndrome, %

90

<1

100

1

110

3

120

5

130

9

140

16

150

25

160

40

170

54

180

70

190

80

200

88

210

93

220

96

230

98

240

99

250

>99

Reference: 1. Fardet L, Galicier L, Lambotte O, et al. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol. 2014;66(9):2613-2620. doi:10.1002/art.38690