DIPSS, DIPSS-plus and MIPSS70 (myelofibrosis risk scores)

What is measured: expected survival in primary myelofibrosis, and so the case for a transplant. How: IPSS (2009, at diagnosis) and DIPSS (dynamic, at any time) score age over 65, haemoglobin under 100 g/L, white cells over 25, circulating blasts of 1 percent or more and constitutional symptoms; DIPSS-plus adds an unfavourable karyotype, platelets under 100 and transfusion dependence, giving low, intermediate-1, intermediate-2 and high risk with median survivals of about 15, 6.5, 3 and 1.3 years. MIPSS70, for transplant-age patients, adds high-molecular-risk mutations (ASXL1, SRSF2, EZH2, IDH1, IDH2, U2AF1 Q157), the absence of a type 1 CALR mutation, two or more such mutations and marrow fibrosis grade 2 or more; MIPSS70-plus version 2 adds a very-high-risk karyotype and sex-adjusted haemoglobin; GIPSS is genetics only, and MYSEC-PM serves myelofibrosis after polycythaemia vera or essential thrombocythaemia. Inputs: blood count and film, marrow biopsy with fibrosis grade, karyotype and a myeloid sequencing panel. What a result changes: intermediate-2 or high risk in a fit patient is the indication for allogeneic transplant, the only cure; lower-risk disease is watched or treated for symptoms and spleen with JAK inhibitors (ruxolitinib, fedratinib, pacritinib when platelets are low, momelotinib when anaemic); the scores set trial entry. Where it matters: primary myelofibrosis.