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Molecular Pathology in Clinical Practice pp 135–150Cite as

Coagulation Disorders

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Abstract

Reduced to its simplest form, the vascular system is a series of interconnected biologic conduits filled with fluid moving under pressure. This system is subject to injury and must repair itself in order to avoid exsanguination. This latter function is provided by the hemostatic system. Thus, normal hemostasis is a reparative process and consists of three major mechanisms: (1) vasospasm, which is the main mechanism for controlling bleeding after transection/avulsion injuries of large arteries, arterioles, and veins; (2) platelet plug formation; and (3) the procoagulant system. The procoagulant system consists of the mechanisms by which fluid blood is converted into an insoluble hemostatic thrombus. While inherited or acquired disorders of all three mechanisms can cause clinically abnormal bleeding, this chapter reviews the molecular basis of inherited disorders of the procoagulant system (e. g., hemophilia A and B, von Willebrand disease). Inherited vascular and platelet disorders associated with abnormal bleeding are not discussed here.

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Author information

Authors and Affiliations

  1. Department of Hematology and Internal Medicine, Department of Laboratory Medicine and Pathology, Mayo Clinic College of Medicine, Rochester, MN, 55905, USA

    Rajiv K. Pruthi MBBS

  2. Division of Hematology, Mayo Clinic, Rochester, MN, 55905, USA

    John A. Heit MD (Professor)

Authors
  1. Rajiv K. Pruthi MBBS
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  2. John A. Heit MD
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Editor information

Editors and Affiliations

  1. Department of Pathology and Laboratory Medicine, Weill Medical College of Cornell University and New York Presbyterian Hospital, New York, NY, 10021, USA

    Debra G. B. Leonard MD, PhD (Professor and Vice Chair for Laboratory Medicine) (Professor and Vice Chair for Laboratory Medicine)

  2. Hematology, Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, PA, 19104, USA

    Adam Bagg MD (Director) (Director)

  3. Department of Pathology and Laboratory Medicine, Emory University Hospital, Atlanta, GA, 30322, USA

    Angela M. Caliendo MD, PhD (Vice Chair and Associate Professor) (Vice Chair and Associate Professor)

  4. Northwestern University Feinberg School of Medicine, Evanston, IL, 60201

    Karen L. Kaul MD, PhD (Professor) (Professor)

  5. Department of Pathology and Laboratory Medicine, Evanston Northwestern Healthcare, Evanston, IL, 60201, USA

    Karen L. Kaul MD, PhD (Professor) (Professor)

  6. Molecular Pathology Laboratory, Department of Pathology and Laboratory Medicine, University of Pennsylvania Health System, Philadelphia, PA, 19104, USA

    Vivianna M. Van Deerlin MD, PhD (Director) (Director)

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© 2007 Springer Science+Business Media, LLC

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Pruthi, R.K., Heit, J.A. (2007). Coagulation Disorders. In: Leonard, D.G.B., Bagg, A., Caliendo, A.M., Kaul, K.L., Van Deerlin, V.M. (eds) Molecular Pathology in Clinical Practice. Springer, New York, NY. https://doi.org/10.1007/978-0-387-33227-7_12

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  • DOI: https://doi.org/10.1007/978-0-387-33227-7_12

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-0-387-33226-0

  • Online ISBN: 978-0-387-33227-7

  • eBook Packages: MedicineMedicine (R0)

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