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Cluster-Like Headache Revealing Polycythemia Vera: A Case Report
1Victor Pauchet Clinic, Amiens, France.
Case Reports in Neurology
|June 30, 2020
Summary
Polycythemia vera (PV) can trigger cluster headache (CH)-like pain. Treatments like venesection and oxygen inhalation effectively managed these headaches, unlike standard sumatriptan.
Area of Science:
- Neurology
- Hematology
Background:
- Cluster headache (CH) is a primary headache disorder characterized by severe unilateral head pain.
- Polycythemia vera (PV) is a myeloproliferative neoplasm associated with increased red blood cell mass.
Observation:
- A 44-year-old man presented with CH-like headaches, later diagnosed with PV.
- Headache attacks were severe, unilateral, and lasted approximately 30 minutes, without autonomic symptoms.
- Initial treatments including sumatriptan were ineffective, but venesection and oxygen inhalation provided significant relief.
Findings:
- The patient's PV diagnosis was confirmed by elevated hemoglobin, hematocrit, positive JAK2 mutation, and bone marrow biopsy.
- Venesection led to substantial headache improvement, suggesting a link between blood viscosity and headache severity.
- Oxygen inhalation proved highly effective for acute CH attack management.
Implications:
- This case highlights a potential link between PV and secondary headache disorders, specifically CH-like pain.
- Understanding the pathophysiology may involve hypoxia, nitric oxide, and calcitonin gene-related peptide.
- Effective management strategies may include phlebotomy and oxygen therapy in patients with PV and headache.
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