Risk Stratification: Primary Myelofibrosis

EP: 1.Myeloproliferative Neoplasms: Diagnosing Polycythemia Vera

EP: 2.Risk Stratification and Treatment Considerations in PV

EP: 3.PV Treatment Options and Dosing

EP: 4.The JAK2 Pathway in Polycythemia Vera

EP: 5.Ruxolitinib in PV: The RESPONSE Trial

EP: 6.Ruxolitinib in PV: The RESPONSE-2 Trial

EP: 7.Future Directions in the Treatment of Polycythemia Vera

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EP: 8.Risk Stratification: Primary Myelofibrosis

EP: 9.Treatment Approaches for Myelofibrosis

EP: 10.Myelofibrosis: Treatment Planning and Management

EP: 11.Ruxolitinib for Myelofibrosis: Treatment Considerations

EP: 12.Selecting Therapy to Treat Myelofibrosis

EP: 13.Unmet Needs in Management of Myeloproliferative Neoplasms

Pankit Vachhani, MD: There are several risk stratification schemas for patients with primary myelofibrosis. In the modern treatment landscape, we have been used to using the IPSS [International Prognostic Scoring Score] risk stratification schema. That incorporates 5 different factors into it: the first is peripheral blood blasts, second is the hemoglobin level, third is white blood cell count level, fourth is constitutional symptoms, and fifth is the age of the patient. In addition to that, there is also the DIPSS [Dynamic International Prognostic Scoring System] and DIPSS Plus risk stratification system. What the DIPSS and DIPSS Plus do is allow for a more dynamic assessment of any individual patient’s risk. We typically use the IPSS risk stratification schema at the time of diagnosis. One could also use DIPSS or DIPSS Plus then, but in order to follow the patients after the point of starting treatment, one typically uses DIPSS or DIPSS Plus.

In addition, if someone happens to have the genetic information of a patient at the time of diagnosis, and that genetic information could be the carrier type and the molecular mutations obtained through next-generation sequencing panels, you may want to utilize the MIPSS70 [Mutation-Enhanced International Prognostic Scoring System for Transplantation-Age Patients] or MIPSS70 Plus risk scoring stratification. MIPSS70 incorporates more factors, and in particular, the genetic factors, into the schema. If you have patients who had essential thrombocytosis [ET] or polycythemia vera [PV] that progressed into myelofibrosis—and in doing that, would have secondary myelofibrosis—one may want to consider using the MYSEC-PM [Myelofibrosis Secondary to PV and ET-Prognostic Model] risk stratification schema. This was specifically designed for patients who have post-ET or post-PV myelofibrosis.

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