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Essential thrombocythemia treatment algorithm 2018
Ayalew Tefferi1, Alessandro M Vannucchi2, Tiziano Barbui3
1Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN, USA. tefferi.ayalew@mayo.edu.
Current treatments for essential thrombocythemia (ET) and polycythemia vera (PV) do not cure or prolong survival. A risk-adapted algorithm for ET and PV treatment prioritizes thrombosis prevention with established therapies.
Area of Science:
- Hematology
- Oncology
- Internal Medicine
Background:
- Myeloproliferative neoplasms, essential thrombocythemia (ET) and polycythemia vera (PV), lack curative therapies.
- Current treatments for ET and PV do not demonstrably prolong survival.
- Prognosis for ET and PV is generally favorable, with long median survivals.
Purpose of the Study:
- To present a risk-adapted, point-of-care treatment algorithm for ET and PV.
- To emphasize a "less is more" approach, avoiding unproven novel therapies.
- To guide treatment decisions based on evidence and extensive clinical experience.
Main Methods:
- Identification of three major thrombosis risk factors: history of thrombosis, JAK2/MPL mutations, and advanced age.
- Categorization of ET patients into four risk groups: very low, low, intermediate, and high risk.
- Development of a treatment algorithm based on these risk categories.
Main Results:
- The algorithm stratifies patients based on combined risk factors.
- It guides the selection of appropriate therapies to prevent thrombosis.
- It advocates for established treatments over newer, unproven drugs.
Conclusions:
- A risk-adapted approach is crucial for managing ET and PV.
- Established therapies are effective for thrombosis prevention in ET and PV.
- The algorithm provides a practical tool for clinicians managing these conditions.
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