Updated recommendations on the use of ruxolitinib for the treatment of myelofibrosis

Figures & data

Table 1. Prognostic models in primary myelofibrosis.

IPSS [7]	DIPSS [8]	DIPSS-plus [9]		MIPSS70 [11]		MIPSS70+ version 2.0 [12]		GIPSS [10]
Clinical variables	Clinical variables	Clinical variables	Genetic variables	Clinical variables	Genetic variables	Clinical variables	Genetic variables	Genetic variables
Age >65 years (1)	Age >65 years (1)	Age >65 years (1)	Unfavorable karyotype (1)	Hemoglobin <10 g/dL (1)	One HMR mutation (1)	Severe anemia (2)	Very high-risk karyotype (4)	Very high-risk karyotype (2)
Hemoglobin <10 g/dL (1)	Hemoglobin <10 g/dL (2)	Hemoglobin <10 g/dL (2)		Leukocytes >25 × 10^9/L (2)	≥2 HMR mutations (2)	Moderate anemia (1)	Unfavorable karyotype (3)	Unfavorable karyotype (1)
Leukocytes >25 × 10^9/L (1)	Leukocytes >25 × 10^9/L (1)	Leukocytes >25 × 10^9/L (1)		Platelets <100 × 10^9/L (2)	Type 1/like CALR absent (1)	Circulating blasts ≥2% (1)	≥2 HMR mutations (3)	Type 1/like CALR absent (1)
Circulating blasts ≥1% (1)	Circulating blasts ≥1% (1)	Circulating blasts ≥1% (1)		Circulating blasts ≥2% (1)		Constitutional symptoms (2)	One HMR mutation (2)	ASXL1 mutation (1)
Constitutional symptoms (1)	Constitutional symptoms (1)	Constitutional symptoms (1)		Constitutional symptoms (1)			Type 1/like CALR absent (2)	SRSF2 mutation (1)
		Platelets <100 × 10^9/L (1)		Bone marrow fibrosis grade ≥2 (1)				U2AF1Q157 mutation (1)
		Transfusion need (1)

Notes: DIPSS, dynamic IPSS; GIPSS, genetically-inspired prognostic scoring system; HMR, high molecular risk; IPSS, international prognostic scoring system; MIPSS70, mutation-enhanced international prognostic scoring system for transplant-age patients; MIPSS70+, karyotype-enhanced MIPSS70. Severe anemia is defined as hemoglobin levels <8 g/dL in women and <9 g/dL in men and moderate anemia as hemoglobin levels 8–9.9 g/dL in women and 9–10.9 g/dL in men.

Risk categories based on patients’ risk scores according to each prognostic system and the expected median survival for all age categories (IPSS, DIPSS and DIPSS-plus) or for patients ≤70 years of age (MIPSS70, MIPSS70+ and GIPSS):

IPSS: low (risk score, 0; median survival, 11.3 years); intermediate-1 (1; 7.9 years); intermediate-2 (2; 4.0 years); high (≥3; 2.3 years).

DIPSS: low (0; not reached); intermediate-1 (1–2; 14.2 years); intermediate-2 (3–4; 4 years); high (5–6; 1.5 year).

DIPSS-plus: low (0; 15.4 years); intermediate-1 (1; 6.5 years); intermediate-2 (2–3; 2.9 years); high (≥4; 1.3 years).

MIPSS70: low (0–1; 27.7 years); intermediate (2–4; 7.1 years); high (≥5; 2.3 years)

MIPSS70+ version 2.0: very low (0; not reached); low (1–2; 16.4 years); intermediate (3–4; 7.7 years); high (5–8; 4.1 years); very high (≥9; 1.8 years).

GIPSS: low (0; 26.4 years); intermediate-1 (1; 8.0 years); intermediate-2 (2; 4.2 years); high (≥3; 2 years).

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