Myelofibrosis Prognostic Models

Myelofibrosis is a clonal myeloproliferative neoplasm with stem cell driven bone marrow fibrosis, ineffective hematopoiesis, splenomegaly, and constitutional symptoms. Risk stratification has evolved from clinical models to molecular and integrated systems that capture the heterogeneous course ranging from indolent disease over years to rapid leukemic transformation.

IPSS at diagnosis uses age above sixty five, hemoglobin below ten, leukocytes above twenty five thousand, blasts above one percent, and constitutional symptoms. DIPSS adds dynamic application during follow up. DIPSS plus incorporates unfavorable cytogenetics, transfusion dependence, and platelets below one hundred thousand. MIPSS70 integrates high molecular risk mutations ASXL1, EZH2, IDH1/2, SRSF2 with bone marrow fibrosis grade.

GIPSS bases risk only on cytogenetics with very high risk including monosomal karyotype, inversion three, isochromosome 17q, and select mutations such as triple negative or high molecular risk. Categories direct treatment from observation through ruxolitinib for spleen and symptom control to fedratinib, momelotinib, or pacritinib in cytopenic disease, and allogeneic transplant for intermediate two and higher categories with acceptable performance status.