From the Guidelines
The International Prognostic Scoring System (IPSS) is crucial for risk stratification in myelofibrosis (MF) patients, particularly when assessing thrombosis risk, as it helps identify patients who may benefit from more aggressive management strategies to reduce morbidity and mortality. Patients with higher IPSS scores generally have increased thrombotic risk, especially those with a history of previous thrombosis 1. For thrombosis management in MF, low-dose aspirin (81-100mg daily) is recommended for most patients, particularly those with a history of thrombosis or high-risk features, as it has been shown to reduce the risk of thrombotic events 1.
When considering the management of thrombosis in MF, it is essential to weigh the risks and benefits of different treatment strategies, prioritizing those that minimize morbidity and mortality. Cytoreductive therapy, such as hydroxyurea, may be considered for patients with higher thrombotic risk or previous arterial events, as it can help reduce the risk of thrombosis while also managing other symptoms of the disease 1. Additionally, regular monitoring of blood counts is essential, as MF patients often have concurrent thrombocytopenia, which increases bleeding risk 1.
In terms of specific treatment strategies, the use of direct oral anticoagulants (DOACs) like apixaban or rivaroxaban may be considered for patients with venous thromboembolism, as they have been shown to be effective in reducing the risk of recurrent thrombotic events 1. However, the decision to use these agents should be made on a case-by-case basis, taking into account the individual patient's risk factors and medical history. Ultimately, the goal of treatment should be to minimize morbidity and mortality, while also improving quality of life for patients with MF.
From the Research
IPSS and Thrombosis in Myelofibrosis
- The International Prognostic Scoring System (IPSS) is used to predict the survival of patients with myelofibrosis (MF) 2.
- Thrombotic events in MF are about as common as in essential thrombocytemia (ET) but less common than in polycythemia vera (PV) 3.
- The risk of thrombosis and hemorrhage in patients with MF is influenced by various factors, including the presence of prefibrotic primary MF (PMF) and the type of mutations present 3, 4, 5.
- The Dynamic International Prognostic Scoring System (DIPSS) and DIPSS Plus are also used to predict the survival of patients with MF and to guide therapeutic decisions 2.
Risk Factors for Thrombosis in Myelofibrosis
- The presence of JAK2, CALR, or MPL mutations can increase the risk of thrombosis in patients with MF 4, 5.
- Other risk factors for thrombosis in MF include the presence of SRSF2, ASXL1, and U2AF1-Q157 mutations, as well as very high-risk abnormalities such as -7, inv (3), i(17q), +21, +19, 12p-, and 11q- 4, 5.
- The type of CALR mutation present can also influence the risk of thrombosis, with type 1/like CALR mutation associated with superior survival 5.
Management of Thrombosis in Myelofibrosis
- The management of thrombosis in MF typically involves the use of JAK2 inhibitors, such as ruxolitinib, fedratinib, and pacritinib, which can help to reduce the risk of thrombosis and improve symptoms 6, 4, 5.
- Other treatments, such as hydroxyurea and splenectomy, may also be used to manage thrombosis in MF 4, 5.
- New agents, alone or in combination with ruxolitinib, are currently being investigated for the treatment of MF and may offer improved management of thrombosis in the future 4, 5.