Hemophilia care in China: review of care for 417 hemophilia patients from 11 treatment centers in Shanxi Province

Abstract

Aims: We analyzed the clinical features of 417 patients with hemophilia from 11 Hemophilia Treatment Centers in Shanxi Province (SP) in China. Methods: We used data collected in the national registry of hemophilia A and hemophilia B in SP from January 2010 to December 2013. Results: Ratio of hemophilia A:hemophilia B patients was 5:1, of which 48% (200/417) were severe, 31% (129/417) moderate and 21% (88/417) mild. Episodes of joint bleeding occurred in 73% (305/417) of patients. Only 4% (15/417) of patients received tertiary prophylaxis. Three percent of patients (2/72) were hepatitis B virus-Ab positive, and 7% (5/72) of patients were hepatitis C virus-Ab positive. The incidence of inhibitors was 6% (11/182). Conclusion: The ability to manage hemophilia in SP remains suboptimal. However, due to limited data, the evaluation and extrapolation of large hemophilia populations in SP are restricted, therefore, further studies with a large cohort are needed.

Keywords:

• China
• clotting
• hemophilia
• inhibitors
• transfusion-transmitted infections

Acknowledgements

We would like to thank M-C Poon (Departments of Medicine, Pediatrics and Oncology, University of Calgary, Canada) for critically reviewing the manuscript. Finally, we would like to thank the Novo Nordisk Hemophilia Foundation to improve access to care in the Shanxi Province. The Novo Nordisk Hemophilia Foundation also supported some costs of inhibitor detection and genetic testing in persons with hemophilia in Shanxi Province. We wish to thank the patients, their families and the clinical personnel who participated in this study.

Financial & competing interests disclosure

This study was supported by research grants from the National Natural Science Foundation of China (81270587), the Shanxi Scholarship Council of China (2009 key 7) and the National Public Health Grand Research Foundation (201202017). The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Key issues

• Individuals with severe hemophilia may present with frequent and spontaneous bleeding episodes in joints or soft tissue, which may eventually cause sufficient levels of pain and swelling to severely limit physical mobility.

• China’s population is over 1.355 billion, there are an estimated 100,000 with hemophilia, and about 4000 people have hemophilia in Shanxi, an underdeveloped province in northern China.

• This retrospective study used data collected in the national registry from cases with hemophilia A or hemophilia B in Shanxi Province (SP) from January 2010 to December 2013. Ultimately, 417 subjects were enrolled in the study. Registry contains information of demographics, symptoms, treatment and laboratory testing.

• Ratio of hemophilia A:hemophilia B patients was 5:1, of which 48% (200/417) were severe, 31% (129/417) moderate and 21% (88/417) mild. Episodes of joint bleeding occurred in 73% (305/417) of patients. Only 4% (15/417) of patients received tertiary prophylaxis. Three percent of patients (2/72) were hepatitis B virus-Ab positive, and 7% (5/72) of patients were hepatitis C virus-Ab positive. The incidence of inhibitors was 6% (11/182). The ability of organizations in SP to diagnose, treat and manage hemophilia was suboptimal.

• Compared with more advanced regions, the condition of persons with hemophilia in SP is worrisome. SP should combine expertise in hemophilia care from ‘developed’ countries to facilitate effective multidisciplinary and multi-sectoral collaborations that will minimize joint disease and maximize quality of life.This summary of the state of hemophilia care and the hemophilia patient population in an underserviced part of China may be a useful addition to the global discussion regarding availability of resources, development of expertise and overall quality improvement of care in this population.