cliot (version 1.0.0)

hscore_hemophagocytic_syndrome: 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)

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.

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).

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)