Genetically Inspired International Prognostic Scoring System (GIPSS) for Primary Myelofibrosis in Adults

 

Karyotype classification:*
		Very high risk (VHR) (2 points)
		Unfavorable (1 point)
		Favorable (0 points)
Driver mutations:
		Absence of type 1-like CALR (1 point)
High molecular risk (HMR) mutations:**
		ASXL1 mutation (1 point)
		SRSF2 mutation (1 point)
		U2AF1 Q157 mutation (1 point)

 
Important: Inputs must be complete to perform calculation.

 

Total Criteria Point Count:
 

 

Interpretation

 

GIPSS score (points)	Risk group	5-year overall survival	Median overall survival (years)
0	Low risk	94%	26.4
1	Intermediate risk - 1	73%	10.3
2	Intermediate risk - 2	40%	4.6
3 to 6	High risk	14%	2.6

 

Notes

• ^*Classification of karyotypes:
  • VHR: Single or multiple abnormalities of -7, i(17q), inv(3)/3q21, 12p-/12p11.2, 11q-/11q23, or other autosomal trisomies not including +8/+9 (eg, +21, +19).
  • Favorable: Normal karyotype or sole abnormalities of +9, 13q-, 20q-, chromosome 1 translocation/duplication, or sex chromosome abnormality including -Y.
  • Unfavorable: All other abnormalities.
• ^**Classification of HMR mutations:
  • ASXL1 mutation = 1 point
  • SRSF2 mutation = 1 point
  • U2AF1 Q157 mutation = 1 point

 

References

1. Tefferi A, Guglielmelli P, Nicolosi M, et al. GIPSS: genetically inspired prognostic scoring system for primary myelofibrosis. Leukemia. 2018; 32:1631. PubMed ID: 29654267