title: Diagnosis and management of von Willebrand disease in China.
abstract: In China, the care of patients with a bleeding disorder is not organized into designated centers with a national protocol. However, currently developed organizations of hematologists and hemophilia patients are beginning to ensure better diagnosis, treatment, and care of affected patients. The diagnosis of von Willebrand disease (VWD) in China is still at an early stage. Misdiagnosis of the disease is difficult to avoid, and there is a need to improve our current diagnostic strategy. Our data show that assessment of von Willebrand factor (VWF) antigen levels is pivotal in ranking VWD clinical severity for replacement therapy. Treatment choices for VWD in China are currently limited to replacement therapy and antifibrinolytic drugs. Most VWD patients in China do not need replacement therapy. New phenotypic assays and genetic testing of VWF for research purposes have developed with results published in the Chinese medical literature in the last few years, and many are practical and feasible for diagnostic application. However, more efforts are needed for their widespread use for precise VWD diagnosis in China.
authors.affiliation: State Key Laboratory of Experimental Hematology, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, Peoples Republic of China.
authors.forename: Jianhui
authors.initials: J
authors.lastname: Xu
authors.forename: Zhiqiang
authors.initials: Z
authors.lastname: Yu
authors.forename: Lei
authors.initials: L
authors.lastname: Zhang
authors.forename: Changgeng
authors.initials: C
authors.lastname: Ruan
authors.forename: Renchi
authors.initials: R
authors.lastname: Yang
mesh_terms: D000933:Antifibrinolytic Agents / Q000627:therapeutic use; D002681:China; D005820:Genetic Testing; D006801:Humans; D009154:Mutation; D014842:von Willebrand Diseases / Q000175:diagnosis* / Q000188:drug therapy* / Q000235:genetics; D014841:von Willebrand Factor / Q000235:genetics
chemical_list: D000933:Antifibrinolytic Agents; D014841:von Willebrand Factor