Thrombosis risk categories for patients with MF

·      Thrombotic risks for patients with MF are numerous and have been incorporated into several ‘prognostic scoring systems’. Current MF prognostic scoring systems are:

o   Mutation-enhanced International Prognostic Scoring System developed for transplant-age patients ≤70 years of age (MIPSS70) and the MPISS70+ which includes additional cytogenetic information. 

·      World Health Organisation (WHO includes six clinical risk variables and two genetic variables:

o   Haemoglobin <10 g/dL

o   White blood cells >25´109/L

o   Platelets <100´109/L

o   Circulating abnormal cells ≥2%

o   Bone marrow fibrosis grade ≥2

o   Clinical symptoms

o   Five high-risk mutations: 

–      ASXL1 (Additional Sex Combs Like-1)

–      SRSF2 (Serine and Arginine Rich Splicing Factor 2) 

–      EZH2 (Enhancer of zeste homolog 2)

–      IDH1 (Isocitrate dehydrogenase 1)

–      IDH2 (Isocitrate dehydrogenase 2)

o   One favourable mutation (CALR type 1/like). 

o   MIPSS70 risk factors were used to compose three risk categories:

–      Low

–      Intermediate

–      High

·      MIPSS70+ includes the same list of mutations but only three clinical risk factors which are:

o   Haemoglobin <10 g/dL

o   Circulating abnormal cells ≥2%

o   Clinical symptoms

o   MIPSS70+ includes a two-tiered cytogenetic risk variable (unfavourable v favourable). 

o   MIPSS70+ risk factors are combined to create 4 risk categories for patients with MF:

–      Low

–      Intermediate

–      High

–      Very high·       Recent research into the genetics of MF has resulted in the development of MIPSS70+ version 2.0 that addresses the fact that MIPSS70+ does not include the additional prognostic distinction between very-high-risk

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