Post-partum hemorrhage in women with rare bleeding disorders

  • Flora Peyvandi
    Corresponding author. Flora Peyvandi, MD, PhD. Angelo Bianchi Bonomi Haemophilia and Thrombosis Centre, Via Pace, 9, 20122, Milan, Italy. Fax: +39 02 54100125
    Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Luigi Villa Foundation, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico and Università degli Studi di Milano, Milan, Italy
    Search for articles by this author
  • Marzia Menegatti
    Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Luigi Villa Foundation, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico and Università degli Studi di Milano, Milan, Italy
    Search for articles by this author
  • Simona Maria Siboni
    Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Luigi Villa Foundation, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico and Università degli Studi di Milano, Milan, Italy
    Search for articles by this author
      This paper is only available as a PDF. To read, Please Download here.


      Post-partum hemorrhage (PPH) accounts for a substantial fraction of maternal deaths in the general population. Among all women, however, those affected with rare bleeding disorders (RBDs) represent a particular group since to usual bleeding symptoms, they are likely to experience bleedings associated to obstetrical and gynaecological problems. Pregnancy and childbirth, two important stages in the life of a woman, pose a special clinical challenge in women with RBDs, since information about these issues are really scarce and limited to few case reports. These data show that all women with RBDs, except for FXI deficiency, have to be considered potentially at risk for developing PPH, therefore they should be monitored carefully during and immediately after pregnancy. The implication is that women with bleeding disorders may require prophylaxis and/or close observation for several weeks and should be followed by a multidisciplinary team including expertises such as laboratory haematologist, obstetrician-gynaecologist, anaesthesiologist, family physician, and laboratory technician.


      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Thrombosis Research
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Ross M
        Placental and fetal physiology.
        in: Gabbe S Niebyl J Simpson J Obstetrics: normal and problem pregnancies. Churchill Livingstone, New York2002: 37-62
        • Cunningham FG
        • Gant NF
        • Leveno KG
        • Gilstrap LC
        • Haunt JC
        • Wenstrom KD
        Williams Obstetrics. 21st edn. McGraw-Hill, New York2001
        • Ljaiya MA
        • Aboyeji AP
        • Abubaker D
        Analysis of 348 consecutive cases of primary postpartum haemorrhage in a tertiary hospital in Nigeria.
        J Obstet Gynecol. 2003; 23: 374-377
        • Bais JMJ
        • Eskes M
        • Pel M
        • Bonsel GJ
        • Bleker OP
        Postpartum haemorrhage in nulliparous women: incidence and risk factors in low and high risk women A Dutch population-based cohort study based on standard (>500 mL) and severe (>1000 mL) postpartum haemorrhage.
        Eur J Obstet Gynecol Reprod Biol. 2004; 115: 166-172
        • Chang J
        • Elam-Evans LD
        • Berg CJ
        • et al.
        Pregnancy-related mortality surveillance – United States, 1991-1999.
        MMWR Surveill Summ. 2003; 52: 1-8
      1. Health Canada. Special Report on Maternal Mortality and Severe Morbidity in Canada Enhanced Surveillance: The Path to Prevention, Ottawa, Canada: Minister of Public Works and Government Services Canada, 2004. accessed 20 September 2010.

        • Cameron CA
        • Roberts CL
        • Olive EC
        • Ford JB
        • Fischer WE
        Trends in postpartum hemorrhage.
        Aust N Z J Public Health. 2006; 30: 151-156
        • Knight M
        • Callaghan WN
        • Berg C
        • et al.
        Trends in postpartum hemorrhage in high resource countries: a review and recommendations from the International Postpartum Hemorrhage Collaborative Group.
        BMC Pregnancy and Childbirth. 2009; 9: 55-65
        • Mannucci PM
        • Duga S
        • Peyvandi F
        Recessively inherited coagulation disorders.
        Blood. 2004; 104: 1243-1252
        • Peyvandi F
        • Palla R
        • Menegatti M
        • Mannucci PM
        Introduction: rare bleeding disorders: general aspects of clinical features, diagnosis, and management.
        Semin Thromb Hemost. 2009; 35: 349-355
        • Peyvandi F
        • Palla R
        • Menegatti M
        European Registry of Rare Bleeding Disorders.
        Hematology Education: the education programme for the annual congress of the European Hematology Association. 2010; 4: 43
        • James AH
        More than menorrhagia: a review of the obstetric and gynaecological manifestations of bleeding disorders.
        Haemophilia. 2005 Jul; 11: 295-307
        • Stirling Y
        • Woolf L
        • North WR
        • et al.
        Haemostasis in normal pregnancy.
        Thromb Haemost. 1984; 52: 176-182
        • Bremme KA
        Haemostatic changes in pregnancy.
        Best Pract Res Clin Haematol. 2003; 16: 153-168
        • Sie P
        • Caron C
        • Azam J
        • et al.
        Reassessment of von Willebrand factor (VWF), VWF propeptide, factor VIII:C and plasminogen activator inhibitors 1 and 2 during normal pregnancy.
        Br J Haematol. 2003; 121: 897-903
        • Haverkate F
        • Samama M
        Familial dysfibrinogenemia and thrombophilia. Report on a study of the SSC Subcommittee on Fibrinogen.
        Thromb Haemost. 1995; 73: 151-161
        • Goodwin TM
        Congenital hypofibrinogenemia in pregnancy.
        Obstet Gynecol Surv. 1989; 44: 157-161
        • Noia G
        • De Carolis S
        • De Stefano V
        • et al.
        Factor V deficiency in pregnancy complicated by Rh immunization and placenta previa. A case report and review of the literature.
        Acta Obstet Gynecol Scand. 1997; 76: 890-892
        • Rizk DE
        • Castella A
        • Shaheen H
        • Deb P
        Factor VII deficiency detected in pregnancy: a case report.
        Am J Perinatol. 1999; 16: 223-226
        • Romagnolo C
        • Burati S
        • Ciaffoni S
        • et al.
        Severe factor X deficiency in pregnancy: case report and review of the literature.
        Haemophilia. 2004; 10: 665-668
        • Catanzarite VA
        • Novotny WF
        • Cousins LM
        • Schneider JM
        Pregnancies in a patient with congenital absence of prothrombin activity: case report.
        Am J Perinatol. 1997; 14: 135-138
        • Peyvandi F
        • Mannucci PM
        Rare coagulation disorders.
        Thromb Haemost. 1999; 82: 1207-1214
        • Salomon O
        • Steinberg DM
        • Tamarin I
        • et al.
        Plasma replacement therapy during labor is not mandatory for women with severe factor XI deficiency.
        Blood Coagul Fibrinolysis. 2005; 16: 37-41
        • Ichinose A
        • Asahina T
        • Kobayashi T
        Congenital blood coagulation factor XIII deficiency and perinatal management.
        Curr Drug Targets. 2005; 6: 541-549
        • Marchant S
        • Alexander J
        • Garcia J
        • Ashurst H
        • Alderdice F
        • Keene J
        A survey of women's experiences of vaginal loss from 24 hours to three months after childbirth (the BLiPP Study).
        Midwifery. 1999; 15: 72-81
        • Visness CM
        • Kennedy KI
        • Ramos R
        The duration and character of postpartum bleeding among breast-feeding women.
        Obstet Gynecol. 1997; 89: 159-163
        • Dahlman T
        • Hellgren M
        • Blombäck M
        Changes in blood coagulation and fibrinolysis in the normal puerperium.
        Gynecol Obstet Invest. 1985; 20: 37-44
        • 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
        • El-Refaey H
        • Rodeck C
        Post-partum haemorrhage: definitions, medical and surgical management. A time for change.
        Br Med Bull. 2003; 67: 205-217
        • Demers C
        • Derzko C
        • David M
        • Douglas J
        • Society of Obstetricians and Gynaecologists of Canada
        Gynaecological and obstetric management of women with inherited bleeding disorders.
        Int J Gynaecol Obstet. 2006; 95: 75-87
        • Kadir R
        • Chi C
        • Bolton-Maggs P
        Pregnancy and rare bleeding disorders.
        Haemophilia. 2009; 15: 990-1005