Acquired von willebrand syndrome in patients with philadelphia-negative myeloproliferative neoplasm

Ik-Chan Song; Sora Kang; Myung-Won Lee; Hyewon Ryu; Hyo-Jin Lee; Hwan-Jung Yun; Deog-Yeon Jo

doi:10.5045/br.2023.2022218

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자료유형: 학술저널

저자정보: Ik-Chan Song (Division of Hematology/Oncology Department of Internal Medicine Chungnam National University College of Medicine Daejeon Korea) Sora Kang (Division of Hematology/Oncology Department of Internal Medicine Chungnam National University College of Medicine Daejeon Korea) Myung-Won Lee (Division of Hematology/Oncology Department of Internal Medicine Chungnam National University College of Medicine Daejeon Korea) Hyewon Ryu (Division of Hematology/Oncology Department of Internal Medicine Chungnam National University College of Medicine Daejeon Korea) Hyo-Jin Lee (Division of Hematology/Oncology Department of Internal Medicine Chungnam National University College of Medicine Daejeon Korea) Hwan-Jung Yun (Division of Hematology/Oncology Department of Internal Medicine Chungnam National University College of Medicine Daejeon Korea) Deog-Yeon Jo (Division of Hematology/Oncology Department of Internal Medicine Chungnam National University College of Medicine Daejeon Korea)

저널정보: 대한혈액학회 Blood Research Blood Research Vol.58 No.1

발행연도: 2023.3

수록면: 42 - 50 (9page)

DOI: 10.5045/br.2023.2022218

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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.

#Myeloproliferative neoplasm #Essential thrombocythemia #Polycythemia vera #Primary myelofibrosis #Acquired von Willebrand syndrome

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