Pediatric Polycythemia Treatment & Management

Updated: Jun 13, 2023
  • Author: May C Chien, MD; Chief Editor: Lawrence C Wolfe, MD  more...
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Approach Considerations

Treatments for primary polycythemia include the following:

  • Phlebotomy - The goal of phlebotomy is to maintain normal red cell mass and blood volume, with a target hematocrit level of 42-46% for men and 39-42% for women
  • Hydroxyurea - Hydroxyurea as a myelosuppressive agent is also widely used in high-risk patients with polycythemia vera (ie, >60 y, history of thrombosis) who require cytoreductive therapy, reducing the need for phlebotomy [2]
  • Interferon - Interferon-alpha is effective in eliminating  JAK2V617Fexpression and inducing hematologic remission
  • Tyrosine kinase inhibitors

Phlebotomy is used for symptomatic hyperviscosity in secondary polycythemia.


Medical Care

Primary polycythemia

The goals of therapy are to maximize survival while minimizing the complications of therapy as well as of the disease itself.  For polycythemia vera, phlebotomy and myelosuppressive chemotherapy are the cornerstones of therapy.  Based on clinical trials in adults, these approaches have produced a median survival time of 9-14 years after the beginning of treatment. Current recommendations for treatment of young patients primarily rely on phlebotomy because the thrombosis is far less likely to occur in children and the long-term risks of leukemia over a longer life span are increased.

Secondary polycythemia

Phlebotomy is used for symptomatic hyperviscosity. The goal is to treat the underlying cause of polycythemia.


Surgical Care

Surgery is not typically indicated. Occasionally, splenectomy is performed late in the course of the disease if massive splenomegaly causes adverse effects such as early satiety, anemia, or thrombocytopenia from sequestration. Please note that these patients have a high risk of complications during surgical procedures.



Diet is unrestricted.