Retrospective analysis of 1312 patients with haemophilia and related disorders in a single Chinese institute

Abstract

With 1.3 billion people, China has the largest population in the world, and therefore has the largest population of persons with haemophilia (PWH). As there is no national registry for haemophilia, it is difficult to ascertain how many PWH have actually been diagnosed. Between January 1983 and June 2002, 1312 patients with coagulation disorders were referred to our hospital, and 1190 patients were evaluable. Among them, 1069 (89.8%) patients had haemophilia, 68 had vWD, 20 had factor XI deficiency, 10 had acquired factor VIII inhibitor and 23 had other coagulation disorders. Of the 1069 PWH, 14.7% were unclassified, 38.4% severe, 35.7% moderate and 11.1% mild. If the unclassified cases were excluded, 45.1% were severe, 41.9% moderate and 13.0% mild. Twenty-nine of the 68 vWD patients had vWF:Ag <5%, and subcategorized as type 3 vWD. Because vWF multimer analysis was not performed in our centre, the remaining vWD patients were not subdivided.

Identification and prevalence of von Willebrand disease type 2N (Normandy) in Australia

“…4 [15][16][17][18][19][20][21][22]. In total, type 2N VWD represents 1-2% of all local VWD presentations.…”

“…gations and on the patient selection strategy is clear from the literature. For example, in a retrospective analysis of 1312 patients with hemophilia and related disorders in a single Chinese institute, 68 individuals could be identified as having VWD [15]. However, as the diagnosis of VWD was based only on VWF:Ag and FVIII:C assays, this study could only differentially identify patients with type 3 VWD (29/68 ¼ 42.6%), and the remainder consisted of 'unclassified' individuals.…”

“…Types 2A and 2M are generally the most common presentations, though these are also sometimes underrecognized when available test processes may not permit their differential diagnosis (e.g. Argentina [21], Brazil [22] and China [15]). Types 2B and 2N VWD are less common phenotypes, although it is likely that their prevalence may also be underrecognized, given either the lack of available testing or specific investigations.…”

Influence of medical insurance schemes and charity assistance projects on regular prophylaxis treatment of the boys with severe haemophilia A in China

“…13 Thus, the incidence of haemophilia arthropathy was high, 14,15 and Chinese children suffered from joint deformities and functional disability, resulting in poor quality of life. Fatal intracranial haemorrhage and epilepsy were also present.…”

Diagnosis and Management ofvon Willebrand Disease in China

“…In our retrospective analysis of 1312 patients with coagulation disorders in the Tianjin Hemophilia Center (also the National Hemophilia Data Management Center) from 1983 to 2002, 1069 had a diagnosis of 1 hemophilia. Only 68 patients had the diagnosis of VWD, 3 suggesting that most cases of VWD are not being diagnosed in China.…”