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Published on: October 3, 2018

Polycythaemia rubra vera and myelofibrosis with spinal cord compression

I C Scott1, C H Poynton

  • 1Department of Haematology, University Hospital of Wales, Heath Park, Cardiff, UK. ian.c.scott@btinternet.com

Journal of Clinical Pathology
|April 29, 2008
PubMed

Insights

Spinal cord compression is a rare complication of myelofibrosis and polycythaemia rubra vera (PRV). Combined treatment offers a better prognosis for neurological improvement and survival in these patients.

Area of Science:

  • Hematology
  • Neurology
  • Oncology

Background:

  • Extramedullary hemopoiesis can cause spinal cord compression, a rare complication in myelofibrosis and polycythemia rubra vera (PRV).
  • Optimal treatment strategies for this condition remain uncertain, necessitating a review of existing literature.

Observation:

  • A case of a 69-year-old male with PRV who developed spinal cord compression due to extramedullary hemopoiesis is presented.
  • A systematic review of 21 reported cases (including the current one) of myelofibrosis and PRV with this complication was conducted.

Findings:

  • The mean symptom duration was 7.6 months, with 17 out of 21 patients being male.
  • Neurological improvement was observed in 14 patients, and 12 survived.
  • Combined treatment (irradiation with laminectomy or chemotherapy) resulted in 75% neurological improvement and 100% survival.
  • Single treatments yielded 67% neurological improvement and 33% survival.

Implications:

  • Spinal cord compression in myelofibrosis and PRV carries a high mortality rate.
  • Combined treatment modalities appear to offer a superior prognosis compared to single treatments.
  • This highlights the importance of considering aggressive, combined therapeutic approaches for better patient outcomes.

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