[Headache caused by polycythemia vera. Classification of a headache under the heading of metabolic disturbances]

As polycythemia vera is a myeloproliferative syndrome, it is based on an autonomic increase in the proliferation of all hematopoetic cells--mostly of erythropoesis. An increase in blood viscosity induces disturbed microcirculation, resulting in headaches with clinical symptoms. So far, the headache in polycythemia vera has not been classified.

We would like to demonstrate a case of a female patient aged 58 years who had been complaining of persistent hemicranial right-sided headaches for the last 16 days, alternating between throbbing and stabbing pain as well as a retrobulbar feeling of pressure. Blood chemistry demonstrated pronounced changes: hemoglobin, 20,1 g/dl; hematocrit, 58%; erythrocytes, 6,8 T/l (6,800,000/microl) leucocytes, 14,400/microl; thrombocytes, 543,000/microl A punch biopsy from the pelvic rim showed cell-rich bone marrow, demonstrating a myeloproliferative syndrome suggestive of polycytemia vera.

Following hemodilution, the headache disappeared within 3 days following normalization of blood chemistry: hemoglobin, 14.9 g/dl; hematocrit, 44%; erythrocytes, 5.2 T/l (5,200,000/microl; leucocytes, 13,100/microl. Further diagnosis of the headache by means of MRI of the head, extra and transcranial doppler sonography as well as MRI of the cervical spine did not produce findings with the exception of retrospondylophyth at C5-6. The patient was therefore managed on aspirin 300 mg daily and was given weekly isovolemic bleeding. The headaches did not recur.

We diagnosed a headache caused by polycythemia vera and recommend classification of this headache under the heading of metabolic disturbances, because this classification meets the diagnostic criteria of the international headache classification.