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KMID : 1142020230580010042
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Blood Research
2023 Volume.58 No. 1 p.42 ~ p.50
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Acquired von willebrand syndrome in patients with philadelphia-negative myeloproliferative neoplasm
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Song Ik-Chan
Kang So-Ra
Lee Myung-Won
Ryu Hye-Won
Yun Hwan-Jung
Lee Hyo-Jin
Yun Hwan-Jung
Jo Deog-Yeon
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Abstract
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Background Acquired von Willebrand syndrome (AVWS) has not been investigated in Korean patients with Philadelphia chromosome-negative myeloproliferative neoplasm.
Methods This study analyzed the prevalence at diagnosis and clinical features of AVWS in patients with essential thrombocythemia (ET), polycythemia vera (PV), prefibrotic/early primary myelofibrosis (pre-PMF), or overt PMF (PMF) diagnosed between January 2019 and December 2021 at Chungam National University Hospital, Daejeon, Korea. AVWS was defined as below the lower reference limit (56%) of ristocetin cofactor activity (VWF:RCo).
Results Sixty-four consecutive patients (36 with ET, 17 with PV, 6 with pre-PMF, and 5 with PMF; 30 men and 34 women) with a median age of 67 years (range, 18?87 yr) were followed for a median of 25.1 months (range, 2.6?46.4 mo). AVWS was detected in 20 (31.3%) patients at diagnosis and was most frequent in ET patients (41.4%), followed by patients with pre-PMF (33.3%) and PV (17.6%) patients. VWF:RCo was negatively correlated with the platelet count (r=0.937; P =0.002). Only one episode of minor bleeding occurred in a patient with ET and AVWS. Younger age (＜50 yr) [odds ratio (OR), 7.08; 95% confidence interval (CI), 1.27?39.48; P =0.026] and thrombocytosis (＞600×109/L) (OR, 13.70; 95% CI, 1.35?138.17; P =0.026) were independent risk factors for developing AVWS.
Conclusion AVWS based on VWF:RCo was common in patients with ET and pre-PMF, but less common in patients with PV in the Korean population. Clinically significant bleeding is rare in these patients.
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KEYWORD
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Myeloproliferative neoplasm, Essential thrombocythemia, Polycythemia vera, Primary myelofibrosis, Acquired von Willebrand syndrome
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