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AJP Rep 2013; 03(01): 029-032
DOI: 10.1055/s-0032-1331376
DOI: 10.1055/s-0032-1331376
Management of Pregnancy in a Patient with Severe Hemophilia Type A
Further Information
Publication History
23 April 2012
10 August 2012
Publication Date:
31 December 2012 (online)
Abstract
Hemophilia type A is a rare inherited bleeding disorder with a diversity of clinical manifestations ranging from persistent bleeding after minor trauma, spontaneous deep muscle or joint hemorrhage, to intracranial hemorrhage. As an X-linked disorder, hemophilia is rare in females and therefore there is little experience with pregnancy and no standardized guidelines to prevent bleeding antepartum, at delivery, and postpartum. We report the clinical course and management of a woman with severe hemophilia A who on two occasions had uncomplicated pregnancies and vaginal deliveries at term utilizing bolus recombinant factor VIII concentrate.
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References
- 1 Mannucci PM, Tuddenham EG. The hemophilias—from royal genes to gene therapy. N Engl J Med 2001; 344: 1773-1779
- 2 Cohen AJ, Kessler CM. Hemophilia A and B. In: Kitchens CS, Alving BM, Kessler CM, , eds. Consultative Hemostasis and Thrombosis. 1st ed. Philadelphia, PA: WB Saunders; 2002: 43-56
- 3 Pike GN, Bolton-Maggs PH. Factor deficiencies in pregnancy. Hematol Oncol Clin North Am 2011; 25: 359-378 , viii–ix
- 4 Centers for Disease Control and Prevention. Report of Universal Data Collection. December 31, 2011
- 5 Lusher JM, McMillan CW. Severe factor VIII and factor IX deficiency in females. Am J Med 1978; 65: 637-648
- 6 Lyon MF. Sex chromatin and gene action in the mammalian X-chromosome. Am J Hum Genet 1962; 14: 135-148
- 7 Dhar P, Abramovitz S, DiMichele D, Gibb CB, Gadalla F. Management of pregnancy in a patient with severe haemophilia A. Br J Anaesth 2003; 91: 432-435
- 8 Kadir RA, Economides DL, Braithwaite J, Goldman E, Lee CA. The obstetric experience of carriers of haemophilia. Br J Obstet Gynaecol 1997; 104: 803-810
- 9 Kujovich JL. von Willebrand disease and pregnancy. J Thromb Haemost 2005; 3: 246-253
- 10 Kadir R, Chi C, Bolton-Maggs P. Pregnancy and rare bleeding disorders. Haemophilia 2009; 15: 990-1005
- 11 Chi C, Kadir RA. Management of women with inherited bleeding disorders in pregnancy. The Obstetrician & Gynaecologist 2011; 9: 27-33
- 12 Caliezi C, Tsakiris DA, Behringer H, Kühne T, Marbet GA. Two consecutive pregnancies and deliveries in a patient with von Willebrand's disease type 3. Haemophilia 1998; 4: 845-849
- 13 Lee CA, Chi C, Pavord SR , et al; UK Haemophilia Centre Doctors' Organization. The obstetric and gynaecological management of women with inherited bleeding disorders—review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors' Organization. Haemophilia 2006; 12: 301-336
- 14 Chi C, Lee CA, Shiltagh N, Khan A, Pollard D, Kadir RA. Pregnancy in carriers of haemophilia. Haemophilia 2008; 14: 56-64
- 15 Letsky EA. Haemostasis and epidural anaesthesia. Int J Obstet Anesth 1991; 1: 51-54
- 16 Chi C, Lee CA, England A, Hingorani J, Paintsil J, Kadir RA. Obstetric analgesia and anaesthesia in women with inherited bleeding disorders. Thromb Haemost 2009; 101: 1104-1111
- 17 Varughese J, Cohen AJ. Experience with epidural anaesthesia in pregnant women with von Willebrand disease. Haemophilia 2007; 13: 730-733
- 18 Ljung R, Lindgren AC, Petrini P, Tengborn L. Normal vaginal delivery is to be recommended for haemophilia carrier gravidae. Acta Paediatr 1994; 83: 609-611