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