title: Retrospective analysis of 1,226 Chinese patients with haemophilia in a single medical centre.
abstract: Haemophilia A (HA) and B (HB) are X-linked congenital disorders caused by deficiencies of Factor VIII and FIX. Being the world's most populous country, China potentially has a large population of haemophilia patients. During the last decade, no studies have been published regarding the clinical information of haemophilia in China. A retrospective study was conducted in patients with HA and HB referred to Tianjin Haemophilia Centre between 2002 and 2012. We identified 1,226 males with haemophilia (1,019 HA and 207 HB). The results revealed that activate partial thromboplastin time was negatively correlated plasma factor level of person with haemophilia. Our data did not offer sufficient evidence of any relationship existed between disease severity and risk or site of haemorrhage. There was a trend toward a higher inhibitor incidence induced by plasma-derived factor VIII products, than by recombinant FVIII (rFVIII) alone. It seemed that second generation of rFVIII more likely developed inhibitor, and first generation of rFVIII was nevertheless more closely connected to high-titer inhibitor. We found that delay in diagnosis and blood-borne infections were significantly reduced, while the joint deformity rate did not decrease despite the wide variety of products to choose from in this decade. The development of inhibitor still remains a major challenge in replacement therapy in haemophilia.
authors.affiliation: State Key Laboratory of Experimental Hematology, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 288 Nanjing Road, Tianjin, 300020, People's Republic of China, mindyxuan@gmail.com.
authors.forename: Min
authors.initials: M
authors.lastname: Xuan
authors.forename: Feng
authors.initials: F
authors.lastname: Xue
authors.forename: Rongfeng
authors.initials: R
authors.lastname: Fu
authors.forename: Yanhui
authors.initials: Y
authors.lastname: Yang
authors.forename: Lei
authors.initials: L
authors.lastname: Zhang
authors.forename: Mengsu
authors.initials: M
authors.lastname: Tian
authors.forename: Renchi
authors.initials: R
authors.lastname: Yang
mesh_terms: D000293:Adolescent; D044466:Asian People; D019774:Blood Coagulation Factor Inhibitors / Q000008:administration & dosage* / Q000097:blood*; D002648:Child; D002675:Child, Preschool; D002681:China / Q000453:epidemiology; D005169:Factor VIII / Q000008:administration & dosage*; D006467:Hemophilia A / Q000097:blood / Q000188:drug therapy / Q000453:epidemiology; D006470:Hemorrhage / Q000097:blood / Q000188:drug therapy / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D008297:Male; D010314:Partial Thromboplastin Time; D012189:Retrospective Studies
chemical_list: D019774:Blood Coagulation Factor Inhibitors; C078147:F8 protein, human; D005169:Factor VIII