Figure 2.
Figure 2. (A) Evolution in prognostic assessment: ET. Constructed prior to CALR discovery (*). CALR co-segregates with younger age and absent thrombosis risk, and therefore, does not modify IPSET score (**). Unable to predict MF or AML risk (***). (B) Evolution in prognostic assessment: PV. (C) Evolution in prognostic assessment: MF. The latest proposed MF scoring systems incorporate molecular and genetic information in the assessment (*). The GPSS identified very high (3 points) and high-risk karyotypes (2 points), TN status (2 points), JAK2/MPL-mutated (2 points), type-2/type-2–like CALR-mutated (2 points), ASXL1-mutated (1 point), and SRSF2-mutated (1 point), as independent predictors of shortened survival; these variables were included in this score, along with age >60 years.57 Another system, the Mutation-enhanced IPSS (MIPSS), analyzed 986 PMF patients, identifying age >60, constitutional symptoms, Hb <10 g/dL, platelets <200 × 109/L, TN status (1.5 points), JAK2-mutated or MPL-mutated (0.5 points), ASXL1-mutated (0.5 points), and SRSF2-mutated (0.5 points) status as significant, adverse indicators.58 CV, cardiovascular; DIPSS, dynamic IPSS; GPSS, genetics-based Prognostic Scoring System; pts, patients.

(A) Evolution in prognostic assessment: ET. Constructed prior to CALR discovery (*). CALR co-segregates with younger age and absent thrombosis risk, and therefore, does not modify IPSET score (**). Unable to predict MF or AML risk (***). (B) Evolution in prognostic assessment: PV. (C) Evolution in prognostic assessment: MF. The latest proposed MF scoring systems incorporate molecular and genetic information in the assessment (*). The GPSS identified very high (3 points) and high-risk karyotypes (2 points), TN status (2 points), JAK2/MPL-mutated (2 points), type-2/type-2–like CALR-mutated (2 points), ASXL1-mutated (1 point), and SRSF2-mutated (1 point), as independent predictors of shortened survival; these variables were included in this score, along with age >60 years.57  Another system, the Mutation-enhanced IPSS (MIPSS), analyzed 986 PMF patients, identifying age >60, constitutional symptoms, Hb <10 g/dL, platelets <200 × 109/L, TN status (1.5 points), JAK2-mutated or MPL-mutated (0.5 points), ASXL1-mutated (0.5 points), and SRSF2-mutated (0.5 points) status as significant, adverse indicators.58  CV, cardiovascular; DIPSS, dynamic IPSS; GPSS, genetics-based Prognostic Scoring System; pts, patients.

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