Beyond Hematologic Control: Comprehensive Symptom Management in PV

Opinion
Video

Panelists discuss how in polycythemia vera (PV), when standard cytoreductive therapies fail to control symptoms, targeted treatments such as ruxolitinib for pruritus and splenomegaly, iron supplementation for fatigue, nonsteroidal anti-inflammatory drugs (NSAIDs) for pain, and psychosocial support play a crucial role in improving symptom management and enhancing patient quality of life.

Summary for Physicians: Managing Symptoms Not Controlled by Standard Cytoreductive Therapy in PV

While cytoreductive therapy (eg, hydroxyurea, interferon, ruxolitinib) is effective in controlling hematologic parameters in polycythemia vera (PV), certain symptoms may persist despite these treatments. Addressing these refractory symptoms is critical for improving patient quality of life and overall disease management.

Options for Managing Symptoms Not Controlled by Standard Cytoreductive Therapy:

  1. Pruritus:
  • Ruxolitinib: Particularly effective for PV-associated pruritus, especially in patients with splenomegaly or those who fail to respond to traditional antihistamines.

  • Antihistamines (eg, cetirizine, hydroxyzine): Can be useful for mild cases but may not be sufficient for severe pruritus.

  • UV-B Phototherapy: For refractory cases of pruritus, especially those not responding to pharmacological interventions.
  1. Fatigue:
  • Iron Supplementation: Chronic phlebotomy may lead to iron deficiency, which can exacerbate fatigue. Iron supplementation (oral or intravenous) can help correct this deficiency and improve energy levels.

  • Exercise and Behavioral Interventions: Regular physical activity and cognitive-behavioral strategies for managing fatigue can be beneficial.

  • Stimulants (eg, modafinil): In certain cases, these can be considered to manage persistent fatigue when other strategies fail.
  1. Splenomegaly:
  • Ruxolitinib: An effective treatment option for managing symptomatic splenomegaly, particularly when associated with significant discomfort, early satiety, or weight loss.

  • Interferon: Can be considered in younger patients or those who need an alternative to ruxolitinib, though it may have a slower onset of action.
  1. Bone Pain or Musculoskeletal Discomfort:
  • NSAIDs (eg, ibuprofen, acetaminophen): Can provide relief from mild pain, though long-term use should be monitored for adverse effects.

  • Hydroxyurea Dose Adjustment: If bone pain is related to treatment, adjusting the dose of hydroxyurea or switching therapies may be necessary.
  1. Quality of Life (QOL) Symptoms:
  • Psychosocial Support: Addressing anxiety, depression, and other psychosocial factors through counseling or pharmacotherapy can significantly improve overall QOL in PV patients.

  • Supportive Care: Referral to palliative care or supportive care teams can help manage refractory symptoms and improve patient comfort, especially in advanced stages of disease.

Conclusion: When standard cytoreductive therapy does not fully control symptoms, additional treatments targeting specific issues like pruritus, fatigue, splenomegaly, and pain are necessary. Tailoring these interventions to the individual patient’s needs is key to optimizing symptom management and improving overall quality of life.

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