Factors related to the development of acquired von Willebrand syndrome in patients with essential thrombocythemia and polycythemia vera

Published:December 02, 2016DOI:


      • AVWS was common and associated with higher bleeding rates and higher platelet count.
      • Importantly, most AVWS patients had platelet counts under 1000 × 109/L.
      • Therefore, VWF testing should be integral to the routine assessment of MPN patients.
      • JAK2 V617F is a main driver for the development of AVWS in ET patients.



      We characterized acquired von Willebrand syndrome (AVWS) among essential thrombocythemia (ET) and polycythemia vera (PV) patients.


      A review of patients with ET or PV evaluated for AVWS.


      Of 116 patients with ET, 64 (55%) developed AVWS; of 57 with PV, 28 (49%) developed AVWS. Median platelet counts of ET and PV patients who developed AVWS were 920 × 109/L and 679 × 109/L, respectively (P = 0.01). Of patients who developed AVWS, 69.5% had platelet counts below 1000 × 109/L. Bleeding was more common in patients with AVWS, among both ET and PV patients (P < 0.001). VWF:RCo levels and VWF:RCo/VWF:Ag ratio were lower among JAK2 V617F positive- vs. JAK2 V617F negative- ET patients (P = 0.02 and P = 0.002, respectively); whereas VWF:Ag levels were comparable (P = 0.96). ET patients harboring the JAK2 V617F mutation were more likely to develop AVWS than were calreticulin-positive patients (70.3% vs. 45.7%, P = 0.02), despite lower platelet counts (median 773 vs. 920 × 109/L, P = 0.05). In multivariable analysis, younger age (P = 0.002), platelet count (P < 0.001), hemoglobin level (P = 0.01) and JAK2 V617F mutation (P = 0.01) independently predicted the development of AVWS among ET patients; whereas only platelet count predicted its development among PV patients (P < 0.001).


      Among ET and PV patients, AVWS was common and associated with higher bleeding rates and higher platelet count; nonetheless, most AVWS patients had platelet counts under 1000 × 109/L. Thus, AVWS screening should be included in routine assessment of ET and PV patients. Among ET patients, JAK2 V617F was a main driver for the development of AVWS.


      AVWS (acquired von Willebrand syndrome), ET (essential thrombocythemia), MPN (myeloproliferative neoplasms), PV (polycythemia vera)


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