Amin C, Sharathkumar A, Griest A. Bleeding diathesis and hemophilias. Handb Clin Neurol. 2014;120:1045–59. https://doi.org/10.1016/B978-0-7020-4087-0.00070-X.

Article  PubMed  Google Scholar 

Ljung RC. Intracranial haemorrhage in haemophilia A and B. Br J Haematol. 2008;140(4):378–84. https://doi.org/10.1111/j.1365-2141.2007.06949.x.

Article  PubMed  Google Scholar 

de Tezanos PM, Fernandez J, Perez Bianco PR. Update of 156 episodes of central nervous system bleeding in hemophiliacs. Haemostasis. 1992;22(5):259–67. https://doi.org/10.1159/000216333.

Article  Google Scholar 

Eyster ME, Gill FM, Blatt PM, Hilgartner MW, Ballard JO, Kinney TR. Central nervous system bleeding in hemophiliacs. Blood. 1978;51(6):1179–88.

Article  CAS  PubMed  Google Scholar 

Iorio A, Stonebraker JS, Chambost H, Makris M, Coffin D, Herr C, et al. Data and Demographics Committee of the World Federation of Hemophilia. Establishing the prevalence and prevalence at birth of hemophilia in males: a meta-analytic approach using national registries. Ann Intern Med. 2019;171(8):540–6. https://doi.org/10.7326/M19-1208.

Article  PubMed  Google Scholar 

Kahn ML, Zheng YW, Huang W, Bigornia V, Zeng D, Moff S, et al. A dual thrombin receptor system for platelet activation. Nature. 1998;394(6694):690–4. https://doi.org/10.1038/29325.

Article  CAS  PubMed  Google Scholar 

Kojima H, Newton-Nash D, Weiss HJ, Zhao J, Sims PJ, Wiedmer T. Production and characterization of transformed B-lymphocytes expressing the membrane defect of Scott syndrome. J Clin Invest. 1994;94(6):2237–44. https://doi.org/10.1172/JCI117586.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Carcao MD. The diagnosis and management of congenital hemophilia. Semin Thromb Hemost. 2012;38(7):727–34. https://doi.org/10.1055/s-0032-1326786.

Article  CAS  PubMed  Google Scholar 

Blanchette VS, Key NS, Ljung LR, Manco-Johnson MJ, van den Berg HM, Srivastava A. Subcommittee on Factor VIII, Factor IX and Rare Coagulation Disorders of the Scientific and Standardization Committee of the International Society on Thrombosis and Hemostasis. Definitions in hemophilia: communication from the SSC of the ISTH. J Thromb Haemost. 2014;12(11):1935–9. https://doi.org/10.1111/jth.12672.

Article  CAS  PubMed  Google Scholar 

Raso S, Lambert C, Boban A, Napolitano M, Siragusa S, Hermans C. Can we compare haemophilia carriers with clotting factor deficiency to male patients with mild haemophilia? Haemophilia. 2020;26(1):117–21. https://doi.org/10.1111/hae.13891.

Article  CAS  PubMed  Google Scholar 

Boggio LN, Green D. Acquired hemophilia. Rev Clin Exp Hematol. 2001;5(4):389–404; quiz following 431. https://doi.org/10.1046/j.1468-0734.2001.00049.x.

Article  CAS  PubMed  Google Scholar 

Makris M, Oldenburg J, Mauser-Bunschoten EP, Peerlinck K, Castaman G. Fijnvandraat K; subcommittee on Factor VIII, Factor IX and Rare Bleeding Disorders. The definition, diagnosis and management of mild hemophilia A: communication from the SSC of the ISTH. J Thromb Haemost. 2018;16(12):2530–3. https://doi.org/10.1111/jth.14315.

Article  CAS  PubMed  Google Scholar 

Abraham S, Duncan EM. A review of factor VIII and factor IX assay methods for monitoring extended half-life products in hemophilia A and B. Methods Mol Biol. 2023;2663:569–88. https://doi.org/10.1007/978-1-0716-3175-1_37.

Article  PubMed  Google Scholar 

Peyvandi F, Kenet G, Pekrul I, Pruthi RK, Ramge P, Spannagl M. Laboratory testing in hemophilia: Impact of factor and non-factor replacement therapy on coagulation assays. J Thromb Haemost. 2020;18(6):1242–55. https://doi.org/10.1111/jth.14784.

Article  PubMed  Google Scholar 

•• Zwagemaker AF, Gouw SC, Jansen JS, Vuong C, Coppens M, Hu Q, Feng X, Kim SK, Van der Bom JG, Fijnvandraat K. Incidence and mortality rates of intracranial hemorrhage in hemophilia: a systematic review and meta-analysis. Blood. 2021;138(26):2853–73. https://doi.org/10.1182/blood.2021011849. The above study was the major systematic review and metanalysis conducted to gather all evidence available to estimate ICH burden among hemophilia patients.

Article  CAS  PubMed  Google Scholar 

Kulkarni R, Presley RJ, Lusher JM, Shapiro AD, Gill JC, Manco-Johnson M, et al. Complications of haemophilia in babies (first two years of life): a report from the Centers for Disease Control and Prevention Universal Data Collection System. Haemophilia. 2017;23(2):207–14. https://doi.org/10.1111/hae.13081.

Article  CAS  PubMed  Google Scholar 

Klinge J, Auberger K, Auerswald G, Brackmann HH, Mauz-Körholz C, Kreuz W. Prevalence and outcome of intracranial haemorrhage in haemophiliacs--a survey of the paediatric group of the German Society of Thrombosis and Haemostasis (GTH). Eur J Pediatr. 1999;158(Suppl 3):S162–5. https://doi.org/10.1007/pl00014346.

Article  PubMed  Google Scholar 

Richards M, Lavigne Lissalde G, Combescure C, Batorova A, Dolan G, Fischer K, et al. European Haemophilia Treatment and Standardization Board. Neonatal bleeding in haemophilia: a European cohort study. Br J Haematol. 2012;156(3):374–82. https://doi.org/10.1111/j.1365-2141.2011.08967.x.

Article  CAS  PubMed  Google Scholar 

Ljung RC. Intracranial haemorrhage in haemophilia A and B. Br J Haematol. 2008;140(4):378–84. https://doi.org/10.1111/j.1365-2141.2007.06949.x.

Article  PubMed  Google Scholar 

Nelson MD Jr, Maeder MA, Usner D, Mitchell WG, Fenstermacher MJ, Wilson DA, et al. Prevalence and incidence of intracranial haemorrhage in a population of children with haemophilia. The Hemophilia Growth and Development Study. Haemophilia. 1999;5(5):306–12. https://doi.org/10.1046/j.1365-2516.1999.00338.x.

Article  PubMed  Google Scholar 

• Witmer C, Presley R, Kulkarni R, Soucie JM, Manno CS, Raffini L. Associations between intracranial haemorrhage and prescribed prophylaxis in a large cohort of haemophilia patients in the United States. Br J Haematol. 2011;152(2):211–6. https://doi.org/10.1111/j.1365-2141.2010.08469.x. Nested case-control study investigating risk factors associated with ICH. This was the first study that demonstrated prescribed prophylaxis showed a protective effect against ICH in patients with severe hemophilia.

Article  PubMed  Google Scholar 

Anderst JD, Carpenter SL, Presley R, Berkoff MC, Wheeler AP, Sidonio RF Jr, et al. Relevance of abusive head trauma to intracranial hemorrhages and bleeding disorders. Pediatrics. 2018;141(5):e20173485. https://doi.org/10.1542/peds.2017-3485.

Article  PubMed  Google Scholar 

Nuss R, Soucie JM. Evatt B; Hemophilia Surveillance System Project Investigators. Changes in the occurrence of and risk factors for hemophilia-associated intracranial hemorrhage. Am J Hematol. 2001;68(1):37–42. https://doi.org/10.1002/ajh.1146.

Article  CAS  PubMed  Google Scholar 

• Zanon E, Iorio A, Rocino A, Artoni A, Santoro R, et al. Intracranial haemorrhage in the Italian population of haemophilia patients with and without inhibitors. Haemophilia. 2012;18(1):39–45. https://doi.org/10.1111/j.1365-2516.2011.02611.x. The association of Italian hemophilia centers conducted a retrospective survey from 1987 to 2008 investigating the location of CNS bleeds, pertinent risk factors for ICH, and treatment modalities.

Article  CAS  PubMed  Google Scholar 

Arkin S, Cooper HA, Hutter JJ, Miller S, Schmidt ML, Seibel NL, et al. Activated recombinant human coagulation factor VII therapy for intracranial hemorrhage in patients with hemophilia A or B with inhibitors. Results of the novoseven emergency-use program. Haemostasis. 1998;28(2):93–8. https://doi.org/10.1159/000022418.

Article  CAS  PubMed  Google Scholar 

Franchini M, Girelli D, Olivieri O, Castaman G, Lippi G, Poli G, et al. Tyr2105Cys mutation in exon 22 of FVIII gene is a risk factor for the development of inhibitors in patients with mild/moderate haemophilia A. Haemophilia. 2006;12(4):448–51. https://doi.org/10.1111/j.1365-2516.2006.01297.x.

Article  CAS  PubMed  Google Scholar 

Cho JY, Lee WS, Park YS, Lee SH, Koh JS. Clinical characteristics and prognostic factors in hemophiliacs with intracranial hemorrhage: a single-center, retrospective experience. Indian J Hematol Blood Transfus. 2016;32(4):488–93. https://doi.org/10.1007/s12288-016-0637-3. Epub 2016 Jan 12

Article  PubMed  PubMed Central  Google Scholar 

Eyster ME, Gill FM, Blatt PM, Hilgartner MW, Ballard JO, Kinney TR. Central nervous system bleeding in hemophiliacs. Blood. 1978;51(6):1179–88.

Article  CAS  PubMed  Google Scholar 

Adcock DM, Strandberg K, Shima M, Marlar RA. Advantages, disadvantages and optimization of one-stage and chromogenic factor activity assays in haemophilia A and B. Int J Lab Hematol. 2018;40(6):621–9. https://doi.org/10.1111/ijlh.12877.

Article  CAS  PubMed  Google Scholar 

Schulman S, Varon D, Keller N, Gitel S, Martinowitz U. Monoclonal purified FVIII for continuous infusion: stability, microbiological safety and clinical experience. Thromb Haemost. 1994;72(3):403–7.

Article  CAS  PubMed  Google Scholar 

Martinowitz UP, Schulman S. Continuous infusion of factor concentrates: review of use in hemophilia A and demonstration of safety and efficacy in hemophilia B. Acta Haematol. 1995;94(Suppl 1):35–42. https://doi.org/10.1159/000204025.

Article  PubMed  Google Scholar 

Hay CR, Doughty HI, Savidge GF. Continuous infusion of factor VIII for surgery and major bleeding. Blood Coagul Fibrinolysis. 1996;7(Suppl 1):S15–9.

CAS  PubMed  Google Scholar 

Holme PA, Tjønnfjord GE, Batorova A. Continuous infusion of coagulation factor concentrates during intensive treatment. Haemophilia. 2018;24(1):24–32. https://doi.org/10.1111/hae.13331.

Article  CAS  PubMed  Google Scholar 

Bolton-Maggs PH. Optimal haemophilia care versus the reality. Br J Haematol. 2006;132(6):671–82. https://doi.org/10.1111/j.1365-2141.2005.05952.x.