OBJECTIVES: To describe the prevalence and the treatment status of hemophilia A (HA), hemophilia B (HB) and Von Willebrand disease (VWD) in India, Russia, Taiwan and Turkey based on existing data, and to compare prophylaxis with on-demand therapy. METHODS: We conducted a systematic literature review in Aug, 2011 using PubMed, EMBASE, and Cochrane Library. No retrieval limitation was set. We also carried out general and targeted internet searches of hemophilia related websites. Reference lists of key reviews were hand-searched for further researches. Studies providing data of prevalence, treatment or cost of hemophilia in India, Russia, Taiwan and Turkey were included. Noteexpress 2 was used to manage the literatures. Meta-analysis was done using the generic inverse variance model. RESULTS: Overall, 7 epidemiological and 15 economic studies were included for the analysis. The prevalence of hemophilia in India, Russia, Taiwan and Turkey was 2.27 per 100 000 (HA+HB), 5.12 per 100 000 (HA+HB+VWD), 3.61 per 100 000 (HA) and 4.93 per 100 000 (HA+HB+VWD) respectively. In India, about 15% patients received no treatment, 47.5% patients used previous blood transfusion, 12.5% used cryoprecipitate and 25% used factor concentrate. 37% of patients used prophylaxis in Russia. In Turkey, prophylaxis was superior to on-demand therapy even when given in a twice-weekly period with intermediate concentrates. Annual extra expenditure of below $5000 per case with prophylaxis compared to on-demand therapy would be needed to permit hemophiliac children to achieve similar capacity as unaffected ones. In Taiwan, approximately $2 million per year would be added to the cost of treatment by having all severe HA patients receive secondary prophylaxis instead of on-demand therapy while 12566 bleeding would be prevented. CONCLUSIONS: The prevalence of hemophilia is different among those 4 districts. Hemophiliacs lack adequate treatment generally. It is practical to switch from on-demand therapy to Prophylaxis.