The World Federation of Hemophilia World Bleeding Disorders Registry: insights from the first 10,000 patients Journal Articles uri icon

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abstract

  • BACKGROUND: The prevalence of hemophilia varies globally, with close to 100% of patients diagnosed in high-income countries and as low as 12% diagnosed in lower-income countries. These inequalities in the care of people with hemophilia exist across various care indicators. OBJECTIVES: This analysis aims to describe the clinical care outcomes of patients in the World Bleeding Disorders Registry (WBDR). METHODS: In 2018, the World Federation of Hemophilia developed a global registry, the WBDR, to permit hemophilia treatment centers to collect clinical data, monitor patient care longitudinally, and identify gaps in management and treatment. RESULTS: As of July 18, 2022, 10,276 people with hemophilia were enrolled from 87 hemophilia treatment centers in 40 countries. Nearly half (49%, n = 5084) of patients had severe hemophilia; 99% were male, 85% had hemophilia A, and 67% were from low-middle-income countries. Globally, the age of diagnosis for people with severe hemophilia has improved considerably over the last 50 years, from 82 months (∼7 years) for those born before 1980 to 11 months for those born after 2010, and most prominently, among people with severe hemophilia in low- and low-middle-income countries, the age of diagnosis improved from 418 months (∼35 years) for those born before 1970 to 12 months for those born after 2010. Overall, the age of diagnosis of people with hemophilia in low- and low-middle-income countries is delayed by 3 decades compared to patients in upper-middle-income countries and by 4 decades compared to patients in high-income countries. CONCLUSION: Data reveal large treatment and care disparities between socioeconomic groups, showing improvements when prophylaxis is initiated to prevent bleeding. Overall, care provided in low-income countries lags behind high-income countries by up to 40 years. Limitations in the interpretation of data include risk of survival and selection bias.

authors

  • Coffin, Donna
  • Gouider, Emma
  • Konkle, Barbara
  • Hermans, Cedric
  • Lambert, Catherine
  • Diop, Saliou
  • Ayoub, Emily
  • Tootoonchian, Ellia
  • Youttananukorn, Toong
  • Dakik, Pamela
  • Pereira, Ticiana
  • Iorio, Alfonso
  • Pierce, Glenn F
  • Abdel Mohsen, M
  • Adeyemo, T
  • Ai Sim, G
  • Al-Rahal, N
  • Alexis, C
  • Ali, T
  • Awodu, O
  • Aysarieva, B
  • Aziz, A
  • Barsallo, N
  • Biswas, A
  • Blair, A
  • Blatny, J
  • Borhany, M
  • Castillo, D
  • Catarino, C
  • Chuansumrit, A
  • Coetzee, M
  • Darwish Mohamad Ibrahim, A
  • Diop, S
  • Djenouni, A
  • El Ekiaby, A
  • El Khorassani, M
  • Fawcett, K
  • Ganieva, A
  • Govindan, S
  • Gwarzo, D
  • Hailemariam, S
  • Harper, P
  • Hassan, T
  • Hassan, M
  • Hermans, C
  • Hernandez, F
  • Imran, A
  • John, J
  • Keikhaei, B
  • Kotila, T
  • Liam, C
  • Marhaeni, W
  • Mbanya, D
  • Mekjarusgul, P
  • Meknassi, N
  • Micic, D
  • Mlombe, Y
  • Motusheva, R
  • Munube, D
  • Nagao, A
  • Najmi, S
  • Narayana Pillai, V
  • Narbekov, T
  • Nasution, D
  • Natesirinilkul, R
  • Nchimba, L
  • N’dogomo, M
  • Neme, D
  • Nguyen, P
  • Nguyen, HM
  • Nguyen Thi, M
  • Nigam, RK
  • Njuguna, F
  • Nwagha, T
  • Obeida, A
  • Owusu-Ofori, S
  • Palascak, J
  • Pellegrini, G
  • Philip, C
  • Ping, CL
  • Poudyal, B
  • Rabbani, G
  • Rakoto Alson, OA
  • Razali, H
  • Ruchutrakul, T
  • Ruiz-Saez, A
  • Saengboon, S
  • Salhi, N
  • Satti, M
  • See Guan, T
  • Shah, S
  • Shikuku, T
  • Si Yuan, N
  • Sidarthan, N
  • Siew Looi, T
  • Songthawee, N
  • Sosothikul, D
  • Surapolchai, P
  • Suryani, S
  • Syakira, NA
  • Thevarajah, A
  • Tzong, TJ
  • Udo, C
  • Wong, L
  • Yuguda, S
  • Zafar, T
  • Zaman Miah, M

publication date

  • November 2023