去氨加压素治疗15例血管性血友病患者的临床观察

OBJECTIVE: To assess the significance of DDAVP use in the diagnosis and treatment of VWD. METHODS: An analysis of 15 VWD cases who referred to Hematology Division of First affiliated Hospital of Soochow University and treated with DDAVP from March 2016 to August 2018 was conducted. Efficacy and treatment response of DDAVP were monitored by observations of changes in factor Ⅷ procoagulant (FⅧ∶C) and von Willebrand Factor (VWF) related indicators before and 2 h after DDAVP injection. RESULTS: Of 15 cases with VWD, 7 males and 8 females with a median age of 23 (6-46) years, 7 of 9 type I VWD patients achieved complete response (CR), 1 type 2A VWD case CR, 5 type 3 VWD ones no response (NR). The VWF multimer analysis in 5 patients combined with other plasma VWF values were in accordance with the known diagnosis. CONCLUSION: DDAVP was effective in most type 1 patients, and ineffective in some type 2 and almost all type 3 cases. It was helpful for diagnosis and subsequent treatment planning.