Successful management of intraperitoneal bleeding with platelet apheresis and von Willebrand factor supplementation in a patient with essential thrombocythemia and acquired von Willebrand syndrome

• Published in: Rinshō ketsueki Vol. 58; no. 8; p. 922
• Main Authors: Ueki, Toshimitsu, Takeshige, Kanako, Sumi, Masahiko, Ueno, Mayumi, Ichikawa, Naoaki, Kobayashi, Hikaru
• Format: Journal Article
• Language: Japanese
• Published: Japan 2017
• Subjects: Adult; Blood Platelets; Female; Hemorrhage - etiology; Hemorrhage - therapy; Humans; Thrombocythemia, Essential - complications; von Willebrand Diseases - drug therapy; von Willebrand Diseases - etiology; von Willebrand Factor - therapeutic use; Essential thrombocythemia; VWF-containing concentrates; Acquired von Willebrand syndrome; Platelet apheresis
• ISSN: 0485-1439
• DOI: 10.11406/rinketsu.58.922

A 36-year-old woman with essential thrombocythemia (ET) was admitted to our hospital for acute lower abdominal pain. Given no family history of bleeding disorder, she was diagnosed with acquired von Willebrand syndrome. Despite having a medical history of venous thrombosis, she had never been treated for ET because of her preferences. On admission, CT scan revealed massive hemorrhage in the ascending colon with the leakage of a contrast agent. Furthermore, a delayed enhancement of fluid collection in the Douglas fossa followingcontrast CT indicated bloody ascites. Laboratory data revealed elevated platelets (1,569×10 /µl) and reduced von Willebrand factor (VWF) :RCo (32%) and VWF:Ag (48%). Platelet apheresis was initiated, combined with the infusion of VWF-containing concentrates and cytoreductive therapy with hydroxyurea. Three days after admission, her platelet count decreased to 992×10 /µl after the second round of platelet apheresis. CT scan revealed no hemorrhage, which implied hemostasis. Because of the absence of symptoms, she was discharged 23 days after admission. These results suggest that platelet apheresis, combined with infusion of VWF-containing concentrates and cytoreductive therapy with hydroxyurea, is an effective approach for the treatment of acquired von Willebrand syndrome characterized by emergent bleeding concomitant with ET.