Highlights
- •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.
Abstract
Objective
We characterized acquired von Willebrand syndrome (AVWS) among essential thrombocythemia
(ET) and polycythemia vera (PV) patients.
Methods
A review of patients with ET or PV evaluated for AVWS.
Results
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).
Conclusion
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.
Abbreviations:
AVWS (acquired von Willebrand syndrome), ET (essential thrombocythemia), MPN (myeloproliferative neoplasms), PV (polycythemia vera)Keywords
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Article info
Publication history
Published online: December 02, 2016
Accepted:
November 21,
2016
Received in revised form:
November 15,
2016
Received:
October 7,
2016
Identification
Copyright
© 2016 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
