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Screen for This in Patients with Polycythemia Vera

Eur J Intern Med; ePub 2016 Dec 2; Rottenstreich, et al

Publish date:

Associated von Willebrand syndrome (AVWS) was common, and linked with higher bleeding rates and higher platelet counts in a review that included 173 individuals with either essential thrombocythemia (ET) or polycythemia vera (PV). Investigators found:

  • 55% of patients with ET developed AVWS; nearly half of those with PV did so.
  • Median platelet counts in patients who developed AVWS were 920×109/L (ET) and 679×109/L (PV).
  • 7 in every 10 of those who developed AVWS had platelet counts below 1000×109/L.
  • Bleeding was more common in patients with AVWS.
  • 70% of ET patients with the JAK2 V617F mutation developed AVWS, vs 46% of calreticulin-positive patients.
  • Predictors of AVWS among ET patients included younger age, platelet count, hemoglobin level, and JAK2 V617F mutation.
  • The only predictor among PV patients was platelet count.

The authors suggested routine screening for AVWS in ET and PV patients.

Citation:

Rottenstreich A, Kleinstern G, Krichevsky S, Varon D, Lavie D, Kalish Y. Factors related to the development of acquired von Willebrand syndrome in patients with essential thrombocythemia and polycythemia vera. [Published online ahead of print December 2, 2016]. Eur J Intern Med. doi:10.1016/j.ejim.2016.11.011.

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