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Inherited Bleeding Disorders in Pregnancy: Rare Coagulation Factor Defects

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Disorders of Thrombosis and Hemostasis in Pregnancy

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Abstract

Inherited deficiencies of plasma proteins involved in blood coagulation generally lead to lifelong bleeding disorders. Rare bleeding disorders (RBDs), discussed in this chapter, represent 3–5 % of all the inherited coagulation deficiencies, with prevalence ranging from approximately 1:500,000 to 1:2,000,000 in the general population. Patients affected by bleeding disorders present a wide spectrum of clinical symptoms that vary from a mild or moderate bleeding tendency to significant episodes. Women with inherited bleeding disorders are particularly disadvantaged since, in addition to suffering from general bleeding symptoms, they are also at risk of bleeding complications from regular haemostatic challenges during menstruation, pregnancy and childbirth. Moreover, such disorders pose important problems for affected women due to their reduced quality of life caused by limitations in activities and work, and alteration of their reproductive life. Management of these women is difficult because of considerable inter-individual variation. Furthermore, reliable information on clinical management is scarce, with only a few long-term prospective studies of large cohorts providing evidence to guide diagnosis and treatment.

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Authors and Affiliations

  1. A. Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Cá Granda Ospedale Maggiore Policlinico, and Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano and Luigi Villa Foundation, Milan, Italy

    Flora Peyvandi MD, PhD, Marzia Menegatti PhD & Simona Maria Siboni MD

Authors
  1. Flora Peyvandi MD, PhD
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  2. Marzia Menegatti PhD
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  3. Simona Maria Siboni MD
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Correspondence to Flora Peyvandi MD, PhD .

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Editors and Affiliations

  1. Aberdare Gardens 36, London, NW6 3QA, United Kingdom

    Hannah Cohen

  2. Dept. Obstetrics & Gynaecology, University College Hospital, Chenies Mews 86-96, London, WC1E 6HX, United Kingdom

    Patrick O'Brien

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Peyvandi, F., Menegatti, M., Siboni, S.M. (2012). Inherited Bleeding Disorders in Pregnancy: Rare Coagulation Factor Defects. In: Cohen, H., O'Brien, P. (eds) Disorders of Thrombosis and Hemostasis in Pregnancy. Springer, London. https://doi.org/10.1007/978-1-4471-4411-3_9

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