Factors related to the development of acquired von Willebrand syndrome in patients with essential thrombocythemia and polycythemia vera.
Eur J Intern Med
; 41: 49-54, 2017 Jun.
Article
em En
| MEDLINE
| ID: mdl-27919526
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). VWFRCo levels and VWFRCo/VWFAg ratio were lower among JAK2 V617F positive- vs. JAK2 V617F negative- ET patients (P=0.02 and P=0.002, respectively); whereas VWFAg 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.Palavras-chave
Texto completo:
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Base de dados:
MEDLINE
Assunto principal:
Policitemia Vera
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Doenças de von Willebrand
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Hemorragia
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Trombocitemia Essencial
Idioma:
En
Ano de publicação:
2017
Tipo de documento:
Article