From the Guidelines
Myelofibrosis patients with a median survival expected to be less than 5 years should undergo AlloSCT, despite its high treatment-related mortality and morbidity, as it is the only potentially curative treatment approach. The prognosis of myelofibrosis varies depending on several risk factors, with the median survival ranging from approximately 2 to 11 years after diagnosis 1.
Key Factors Influencing Prognosis
- Age
- Hemoglobin level
- White blood cell count
- Platelet count
- Constitutional symptoms
- Blast percentage
- Specific genetic mutations Scoring systems such as IPSS can help determine the prognosis and identify patients who may benefit from AlloSCT 1.
Treatment Options
- AlloSCT: the only potentially curative option, but carries significant risks
- JAK inhibitors: can improve symptoms and quality of life, but don't significantly alter disease progression Regular monitoring with complete blood counts and physician visits is essential to manage disease progression and prevent complications 1.
From the Research
Myelofibrosis Prognosis
- Myelofibrosis is a rare bone marrow disorder associated with a high symptom burden, poor prognosis, and shortened survival 2.
- The prognosis of myelofibrosis varies depending on the risk score, with intermediate-2 and high-risk groups having a poorer prognosis 3, 4.
- Allogeneic hematopoietic stem cell transplantation (HSCT) is the only curative treatment for myelofibrosis, but it is associated with significant risks and is not suitable for all patients 4, 5.
- Ruxolitinib, a JAK1 and JAK2 inhibitor, is effective in decreasing symptomatic splenomegaly and myelofibrosis-related symptoms, but it is not curative and has no clear disease-altering activity 3, 4, 2, 6.
- The use of ruxolitinib has been shown to improve survival and reduce symptoms in patients with myelofibrosis, but it is not a substitute for HSCT 3, 2.
Treatment Options
- Ruxolitinib is indicated and reimbursed for transplant-ineligible myelofibrosis patients from intermediate-2- and high-risk groups and from the intermediate-1-risk group with splenomegaly 2.
- Allogeneic HSCT is recommended for patients with myelofibrosis who are eligible for transplantation and have a suitable donor 4, 5.
- The decision to use ruxolitinib or HSCT should be based on individual patient characteristics, including risk score, symptoms, and comorbidities 5, 2.
Risk Stratification
- Prognostic risk scores, such as the International Prognostic Scoring System, are used to guide management and predict outcomes in patients with myelofibrosis 4, 6.
- Risk stratification is important to determine the optimal treatment strategy for each patient, taking into account their individual characteristics and comorbidities 5, 2.