cliot (version 1.0.0)

gipss_pmf_score: Genetically Inspired Prognostic Scoring System (GIPSS) for Primary Myelofibrosis

Description

Calculates the GIPSS score to estimate overall survival in patients with Primary Myelofibrosis (PMF). Unlike other scoring systems (e.g., DIPSS), GIPSS relies exclusively on genetic markers (karyotype and mutations) and one clinical variable (platelet count).

Usage

gipss_pmf_score(karyotype_category, platelet_count, asxl1_mutation,
                srsf2_mutation, u2af1_q157_mutation)

Value

A list containing:

GIPSS_Score

The calculated score (Range 0-6).

Risk_Category

Classification (Low, Int-1, Int-2, High).

Median_Overall_Survival

Estimated median survival based on the Mayo Clinic validation cohort.

Arguments

karyotype_category

String. Cytogenetic risk category. "very_high_risk": Single/multiple abnormalities of -7, i(17q), inv(3)/3q21, 12p-/12p11.2, 11q-/11q23, or +21 (+2 points). "unfavorable": Any other abnormal karyotype (+1 point). "favorable": Normal karyotype or sole abnormalities of 13q-, 20q-, +9, chrm 1 translocation/duplication, or sex chromosome abnormality (0 points).

platelet_count

Numeric. Platelet count in 10^9/L. (< 100 x 10^9/L adds +1 point).

asxl1_mutation

Numeric (0 or 1). Presence of ASXL1 mutation. (1 = Yes, +1 point).

srsf2_mutation

Numeric (0 or 1). Presence of SRSF2 mutation. (1 = Yes, +1 point).

u2af1_q157_mutation

Numeric (0 or 1). Presence of U2AF1 Q157 mutation. (1 = Yes, +1 point).

References

Tefferi A, Guglielmelli P, Nicolosi M, et al. GIPSS: genetically inspired prognostic scoring system for primary myelofibrosis. Leukemia. 2018;32(7):1631-1642. doi:10.1038/s41375-018-0107-z

Examples

# Example 1: High Risk
# VHR Karyotype (+2), Platelets 150 (0), ASXL1 (+1)
# Score = 3
gipss_pmf_score("very_high_risk", 150, 1, 0, 0)

# Example 2: Low Risk
# Favorable Karyotype, Platelets 250, No mutations
# Score = 0
gipss_pmf_score("favorable", 250, 0, 0, 0)