HScore for Reactive Hemophagocytic Syndrome
Diagnoses reactive hemophagocytic syndrome based on clinical and laboratory data.
Refer to the text below the calculator for more information about the HScore and the original study.
Reactive hemophagocytic syndrome (RHS) is a rare, life-threatening condition, characterized by fever, pancytopenia, liver failure, coagulopathy, and neurologic symptoms.
Because it does not present with its own combination of clinical, biological or histologic features, differential diagnosis may be difficult to establish against sepsis or hematologic malignancies. The
The HScore has been developed and validated to provide an easy to use diagnostic tool that accounts for patient data in a simple to use model.
| HScore | Probability of hemophagocytic syndrome | HScore | Probability of hemophagocytic syndrome |
| ≤90 | <1% | 171-180 | 54-70% |
| 91-100 | ~1% | 181-190 | 70-80% |
| 101-110 | 1-3% | 191-200 | 80-88% |
| 111-120 | 3-5% | 201-210 | 88-93% |
| 121-130 | 5-9% | 211-220 | 93-96% |
| 131-140 | 9-16% | 221-230 | 96-98% |
| 141-150 | 16-25% | 231-240 | 98-99% |
| 151-160 | 25-40% | ≥241 | >99% |
| 161-170 | 40-54% | - | |
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HScore for Reactive Hemophagocytic Syndrome
Reactive hemophagocytic syndrome (RHS) is a rare, life-threatening condition, characterized by fever, pancytopenia, liver failure, coagulopathy, and neurologic symptoms.
It usually occurs as complication of rheumatic diseases (most notably systemic lupus erythematosus), in patients with lymphoma, serious infection or who have undergone organ transplantation.
Because it does not present with its own combination of clinical, biological or histologic features, differential diagnosis may be difficult to establish against sepsis or hematologic malignancies.
The HSCore developed by Fardet et al. to help diagnose RHS, based on the following clinical and laboratory data:
| HScore item | Answer choices | Pts |
| Known underlying immunosuppression HIV positive or receiving long-term immunosuppressive therapy (i.e., glucocorticoids, cycloSPORINE, azaTHIOprine) |
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 Defined as hemoglobin ≤9.2 g/dL (≤5.71 mmol/L) and/or WBC ≤5,000/mm³ and/or platelets ≤110,000/mm³ |
1 lineage | 0 |
| 2 lineages | 24 | |
| 3 lineages | 34 | |
| Ferritin, ng/mL (or μg/L) | <2,000 | 0 |
| 2,000–6,000 | 35 | |
| >6,000 | 50 | |
| Triglyceride, 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 | |
| Aspartate Aminotransferase (U/L) | <30 | 0 |
| ≥30 | 19 | |
| Hemophagocytosis features on bone marrow aspirate | No | 0 |
| Yes | 35 |
The points associated to the selected answer choices are summed to provide a final score which is then checked against the probability of hemophagocytic syndrome, as per the table below:
| HScore | Probability of hemophagocytic syndrome | HScore | Probability of hemophagocytic syndrome |
| ≤90 | <1% | 171-180 | 54-70% |
| 91-100 | ~1% | 181-190 | 70-80% |
| 101-110 | 1-3% | 191-200 | 80-88% |
| 111-120 | 3-5% | 201-210 | 88-93% |
| 121-130 | 5-9% | 211-220 | 93-96% |
| 131-140 | 9-16% | 221-230 | 96-98% |
| 141-150 | 16-25% | 231-240 | 98-99% |
| 151-160 | 25-40% | ≥241 | >99% |
| 161-170 | 40-54% | - | |
In the original study by Fardet et el. a multicenter cohort of 312 patients was used to construct and validate the HScore.
Ten variables were analysed in association with the diagnosis of RHS and logistic regression was used to calculate the weight of each criterion included in the score. The ten variables were divided as follows: 3 clinical, 5 biologic and 1 cytologic.
The possible number of points assigned to each variable ranged from 0-18 for known underlying immunosuppression to 0-64 for triglyceride level.
In the study cohort, the median HScore was 230 for patients with positive diagnosis, respectively 125 for patients with negative diagnosis.
It was concluded that the HScore is a valid and sensitive model for estimating individual risk of RHS.
A further validation study was undertaken by Debaugnies et al. to check the HScore’s properties in diagnosing hemophagocytic lymphohistiocytosis in a multicenter cohort consisting of adult and pediatric cases of suspected HLH.
References
Original reference
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):2613-20.
Validation
Debaugnies F, Mahadeb B, Ferster A, et al. Performances of the H-Score for Diagnosis of Hemophagocytic Lymphohistiocytosis in Adult and Pediatric Patients. Am J Clin Pathol. 2016; 145(6):862-70.
Specialty: Emergency
Objective: Diagnosis
Year Of Study: 2014
Abbreviation: HScore
Article By: Denise Nedea
Published On: November 10, 2020 · 12:00 AM
Last Checked: November 10, 2020
Next Review: November 10, 2025