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% -

1

Known underlying immunosuppression

HIV positive or receiving long‐term immunosuppressive therapy (i.e., glucocorticoids, cycloSPORINE, azaTHIOprine)
2

Temperature

3

Organomegaly

4

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
5

Ferritin

6

Triglyceride

7

Fibrinogen

8

Aspartate Aminotransferase

9

Hemophagocytosis features on bone marrow aspirate

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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

Last Checked: November 10, 2020

Next Review: November 10, 2025