hscore_hemophagocytic_syndrome: HScore for Reactive Hemophagocytic Syndrome

In cliot: Clinical Indices and Outcomes Tools

HScore for Reactive Hemophagocytic Syndrome

Description

Calculates the HScore to estimate the probability of reactive hemophagocytic syndrome (Secondary HLH) in adult patients. The score assigns points for immunosuppression history, temperature, organomegaly, cytopenias, ferritin, triglycerides/fibrinogen, AST, and bone marrow hemophagocytosis features.

Usage

hscore_hemophagocytic_syndrome(immunosuppression, temp_c, hepatomegaly,
                               splenomegaly, cytopenias_count, ferritin_ng_ml,
                               triglyceride_mg_dl, fibrinogen_mg_dl,
                               ast_u_l, hemophagocytosis_marrow)

Arguments

immunosuppression	Numeric (0 or 1). Known underlying immunosuppression (e.g., HIV, steroid use). (1 = Yes, +18 pts).
temp_c	Numeric. High body temperature in Celsius. < 38.4: 0 pts. 38.4 - 39.4: +33 pts. > 39.4: +49 pts.
hepatomegaly	Numeric (0 or 1). Presence of Hepatomegaly. (1 = Yes).
splenomegaly	Numeric (0 or 1). Presence of Splenomegaly. (1 = Yes). Note: Hepatomegaly OR Splenomegaly = +23 pts. Hepatomegaly AND Splenomegaly = +38 pts.
cytopenias_count	Numeric. Number of cytopenias (Hb <= 9.2 g/dL, WBC <= 5000/mm3, Plt <= 110,000/mm3). 1 lineage: 0 pts. 2 lineages: +24 pts. 3 lineages: +34 pts.
ferritin_ng_ml	Numeric. Serum Ferritin level. < 2000: 0 pts. 2000 - 6000: +35 pts. > 6000: +50 pts.
triglyceride_mg_dl	Numeric. Serum Triglyceride level in mg/dL. (> 1.5 mmol/L or ~133 mg/dL counts towards metabolic criteria).
fibrinogen_mg_dl	Numeric. Serum Fibrinogen level in mg/dL. (<= 2.5 g/L or 250 mg/dL counts towards metabolic criteria). Note: If Triglyceride > 133 mg/dL OR Fibrinogen <= 250 mg/dL, add +64 pts.
ast_u_l	Numeric. SGOT/AST level in U/L. (>= 30: +19 pts).
hemophagocytosis_marrow	Numeric (0 or 1). Hemophagocytosis features on bone marrow aspirate. (1 = Yes, +35 pts).

Value

A list containing:

HScore	The calculated total score (Range 0-337).
HLH_Probability	Estimated probability of having Hemophagocytic Lymphohistiocytosis (HLH). A cutoff of 169 usually yields high sensitivity and specificity.

References

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

Examples

# Example 1: High Probability (HLH Likely)
# Immuno(1), Temp 40(1), Hepatosplenomegaly(2), 3 Cytopenias(3),
# Ferritin 8000(2), High Tri(1), AST 50(1), Marrow Yes(1)
hscore_hemophagocytic_syndrome(1, 40.0, 1, 1, 3, 8000, 200, 200, 50, 1)

# Example 2: Low Probability
# No Immuno, Temp 38.0, No Organomegaly, 1 Cytopenia, Ferritin 500,
# Normal Tri/Fib, AST 20, No Marrow
hscore_hemophagocytic_syndrome(0, 38.0, 0, 0, 1, 500, 100, 300, 20, 0)

cliot documentation built on Dec. 1, 2025, 1:06 a.m.