NCCN Guidelines for Patients® | Myeloproliferative Neoplasms

44 NCCN Guidelines for Patients ® : Myeloproliferative Neoplasms, 2018 Initial treatment There are three treatment goals for MF. The first is to reduce symptoms. Most people have symptoms. Symptoms are often related to an enlarged spleen and anemia. The second treatment goal is to improve blood counts. The third goal is to reduce the chance of MPN progressing to AML. Guide 8 lists the treatment options for MF. Options are based on risk and the presence of symptoms. The methods of assessing risk and symptoms are described next. Risk group There are a few scoring systems for risk. The DIPSS- PLUS ( D ynamic I nternational P rognostic S coring S ystem- PLUS ) is the preferred system during the course of treatment. Your doctor will complete the DIPSS-PLUS. Scores are based on several factors. Blood results are used including white blood cell and platelet counts. Your age is another factor. Scores increase if you need a red blood cell transfusion, have abnormal chromosomes, or have MF-related constitutional symptoms. These symptoms include fevers, night sweats, or weight loss. DIPSS-PLUS total scores range from 0 to 6. Low risk is a score of zero. INT-1 ( int ermediate- 1 ) is a score of 1. INT-2 ( int ermediate- 2 ) is a score of 2 or 3. High risk is a score between 4 and 6. Symptom survey Your treatment will also be planned based on which, if any, symptoms are present. The MPN-SAF Total Symptom Score is advised for assessing symptoms. This survey consists of 10 symptoms. Fatigue is also queried. Each symptom is rated on a scale from 0 to 10. Higher scores point to worse symptoms. Observation Observation is an option for low- and INT-1-risk MF without symptoms. It is a period of testing to watch for changes in cancer status. It is also sometimes called “watch-and wait.” Treatment may be started if symptoms appear. Starting treatment before symptoms appear doesn’t help improve outcomes. Clinical trial Joining a clinical trial is an option for all risk groups. The focus of the trial should be one of the following: treatment to reduce bone marrow fibrosis; treatment to improve cell counts and reduce symptoms; treatment to restore transfusion-independence; or treatment to prevent or delay leukemia. 5 Myelofibrosis Initial treatment Guide 8. Initial treatment options by risk group Low risk - no symptoms Low risk - symptoms INT-1 INT-2 and high risk • Observation • Ruxolitinib • Observation • Allogeneic HCT • Clinical trial • Interferon • Ruxolitinib • Clinical trial • Hydroxyurea • Clinical trial • Ruxolitinib • Clinical trial • Allogeneic HCT • Anemia treatment INT = Intermediate