Myeloproliferative Neoplasms

Part of the series Hematologic Malignancies pp 119-129


Polycythemia Vera and Essential Thrombocythemia: When to Change Therapy – Second-Line Options

  • Alessandro M. VannucchiAffiliated withSection of Hematology, University of Florence Email author 

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Criteria for deciding when to shift a polycythemia vera (PV) or essential thrombocythemia (ET) patient from first-line to second-line therapy have been recently outlined in the management recommendations developed from European LeukemiaNet (ELN) experts. Reasons for second-line therapy are mainly represented by development of refractoriness or intolerance to first-line drugs, in case of high-risk patients already under treatment, or the appearance of any modifications in hematological and clinical manifestations that foresee a worsening of the underlying illness, in case of untreated low-risk subjects. Options for second-line therapy include cytotoxic drugs, interferon, or anagrelide; there is no specific consensus-based evidence on which drug to prefer, or with which priority, and choices mostly reflect the attitude and experience of the physician. Furthermore, cytotoxic drugs other than hydroxyurea used for second-line therapy might raise safety issues concerning their leukemogenic potential and should be used with caution.