top of page
Review Articles





Chinmaya Keshari Sahoo, K.Satyanarayana, D.VenkataRamana

ABSTRACT: Hemophilia is an inherited bleeding disorder where one of the blood clotting proteins is absent or present in a reduced amount. People with Hemophilia, do not bleed faster than anyone else; but will bleed continuously at the normal rate until they are treated. As this is a genetic disorder no complete cure is possible as of now. The available treatment for Hemophilia is by replacing the missing clotting factor in the blood through an intravenous infusion of clotting factor concentrate. Several new technologies are also being implemented to advance Hemophilia, treatment. The present review provides an overview of hemophilia.

KEYWORDS: Hemophilia, mutations, bleeding.


  1. Srivatasva A. editor.Guidelines for management of hemophilia in India.HemophiliaFederation (India), 2003

  2. Bolton-Maggs PH, Pasi KJ. Hemophilias A and B. 2003; 361(9371): 1801-9.

  3. Stone braker JS, Bolton-Maggs PH, Soucie JM, Walker I, Brooker M. A study of variations in the reported Hemophilia-prevalence around the world. 2010; 16: 20–32.

  4. Anwarul KM, Chowdhury YJ. Bangladesh J Child Health 2013; 37 (1) : 27-40.

  5. Somwanshi SB, More VB,HiremathSN,DolasRT,KotadeKB,Gaware VM. Hemophilia-inherited bleeding disorder: an overview,World Journal of Pharmacy and Pharmaceutical Sciences 2014;3(3):596-620

  6. MacFariane RG. An enzyme cascade in the blood clotting mechanism, and its function as a biochemical amplifier, Nature, 1964; 202: 498-9.

  7. Davie EW, Ratnoff OD.Waterfall sequence for intrinsic blood clotting, Science, 1964; 145:1310-2.

  8. Agarwal BRCurrimbhoy ZE. Why do hemophiliacs bleed. Indian Pediatrics1995; 4:505-9.

  9. Peter J.LentingJA, Mourik V, Koen Mertens KT. The life cycle of coagulationfactor VIII in view of its structure and function, blood, 1998; 92(11):3983-3996.

  10. Augusto BF. The factor VIII/Von willebrand factor complex: basicsa and clinical issues,Heamatological/ Journal of hematology, 2003; 88: 1-11.

  11. Ludlam CA, SmithMP, Morfini M, et al.A prospective study of recombinant activated factor VIIadministrated by continuous infusion to inhibitor patients undergoing elective major orthopaedic surgeryapharmacokinetic and efficacy evalution, Br J Heamatol,2003; 120(5):808-13.

  12. Butenas S, Brummel KE, Branda RF et al Mechanism of factor VIIa- dependent coagulation in hemophilia blood, Blood, 2002; 99(3):923-30.

  13. Monroe DM, HoffmanM, Oliver JA,RobertsHR, A possible mechanism of action ofactivated factor VIIindependent of tissue factor. Blood coagulFibrinolysis, 1998; supply 1:S: 15-20.

  14. Mayer SA, BrunNC, Begtrup K, et al, Recombinant activated factor VIIfor acute intracerebralhemorrhage, N Engl J Med, 2005; 352(8):777-85.

  15. Pierce GF,LillicrapD,Pipe SW, Vandendriessche T. Gene therapy, bioengineered clotting factors and novel technologies for hemophilia treatment, J ThrombHaemost, 2007;5:901.

  16. Lorenz J, Martin AH, Christina R, Nicola MW, Mark AK, Anja E. A rapid protocol for the construction and production of high capacity adeno-virus vectors. Nature protocols2009; 4:547-564.

  17. Nathwani AC Niemhuis AW, Davidoff AM. Current status of gene therapy for hemophilia ,currHematol Rep, 2003; 4:319-27.

bottom of page