Analysis of haemophilia case information of Shandong Province in China: Data from 2010 to 2017

Abstract

Haemophilia is an X‐linked haemorrhagic disorder and characterized by deficit or defective functioning of coagulation factors VIII (haemophilia A, HA) or IX (haemophilia B, HB). It is estimated that there are at least 400 000 haemophilia patients worldwide.1 In spite of relatively low prevalence, haemophilia imparts large social and economic burden because of its serious chronic morbidity. Maintaining good haemophilia epidemiological data and disease management strategies is therefore important. Many countries have established a haemophilia or hereditary haemorrhagic disease registration management system.2-5 As a large developing country, China has a population of 1.38 billion and an estimated haemophilia patients over 80 000-120 000.6 The Shandong Hemophilia Treatment Center affiliated to the Shandong Blood Center serves haemophilia patients in the Shandong Province with a population of more than 99 million and 17 prefecture‐level cities, covering an area of 158 000 square kilometres. As one of the six Hemophilia Treatment Centers Collaborative Network of China centres, this centre was approved and established in October 2001 with the support of the Shandong Provincial Department of Health. Since 2009, our centre started a Provincial Hemophilia Registry System as a part of the National Hemophilia Registry System, to collect information on patients with haemophilia and other rare bleeding disorders. This is now the largest provincial registry in China, having registered 2586 patients (haemophilia/rare bleeding disorders) as of December 2017. Analysis of the registry case information will allow a comprehensive understanding of the basic information, clinical features and regional distribution of haemophilia in Shandong Province. The Registry now includes 2091 patients with HA, 362 HB and 133 other rare coagulation defect (Table 1). Most patient diagnoses were confirmed in the central laboratory at the Shandong Blood Center in Jinan, and some patients were diagnosed in other hospitals and registered by our centre. All patients were registered with their ID number, and the ID number is unique. Among haemophilia patients, HA represents 85.3%, haemophilia B 14.8%, similar to those reported by the World Health Organization and the World Federation of Hemphilia.7 The age range of 2453 haemophilia patients was 6 month to 83 years (median 25 years) with the age distribution basically the same as that reported in many other countries.8-10 Our haemophilia patients are mainly children and adolescents, with 64.7% under 30 years of age. Figure1 shows the distribution map of patients with haemophilia in 17 prefecture‐level cities in the province. The names of the 17 prefecture‐level cities and the number of haemophilia cases in the area are shown in the map. The grayer the area in the map, the more cases there are. Heze, Jining, Linyi and Jinan cities have more patients registered, while relatively few patients were registered in Weihai, Yantai, Rizhao and Laiwu cities. The reasons may be related to different levels of medical and healthcare availability, population difference, marriage customs and migration pattern, although it is not easy to obtain relevant evidence. The prevalence of haemophilia in Heze (4.00/100 000) and Dongying (3.47/100 000) are relatively high. This is in contrast to the prevalence of 2.57 per 100 000 for HA and HB in mainland China conducted by the predecessor of Hemophilia Treatment Centers Collaborative Network of China from 1986 to 1989.11 A meta‐analysis of the prevalence of haemophilia in Mainland China by Qu Yanji et al included 22 articles published from 1985 to 2010, showed a weighted combined prevalence of haemophilia A and haemophilia B was 2.8/100 000.12 Taking into account the difficulty of conducting large‐scale epidemiological investigations, further strengthening the management of haemophilia registration management would be needed to master epidemiological and clinical data of haemophilia patients in China as a large country. The haemophilia patients were mainly severe (56.5%) and moderate (30.3%) types. Patients of mild type were less and accounting for only 13.2%. This is in contrast to the higher proportion (36.5%) Accepted: 11 February 2019