Abstract
The vascular system is a series of interconnected biologic conduits filled with fluid moving under pressure. This system is subject to injury, with exsanguination avoided by the functions of the hemostatic system. Normal hemostasis is a reparative process and consists of three major mechanisms: (1) vasospasm, (2) platelet plug formation, and (3) the procoagulant system. While inherited or acquired disorders of all three mechanisms can cause clinically abnormal bleeding, this chapter reviews the molecular basis of the more commonly inherited disorders of the procoagulant system (e.g., hemophilia A and B, and von Willebrand disease) associated with abnormal bleeding.
Original language | English (US) |
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Title of host publication | Molecular Pathology in Clinical Practice |
Subtitle of host publication | Second Edition |
Publisher | Springer International Publishing |
Pages | 203-223 |
Number of pages | 21 |
ISBN (Electronic) | 9783319196749 |
ISBN (Print) | 9783319196732 |
DOIs | |
State | Published - Jan 1 2016 |
Keywords
- Bleeding disorder
- Coagulation
- Diagnostic testing
- F2 gene
- F5 gene
- F8 gene
- F9 gene
- Factor IX deficiency
- Factor V leiden
- Factor VIII deficiency
- Hemophilia A
- Hemophilia B
- Homocystinuria
- Hyperhomocysteinemia
- Molecular testing
- Prothrombin
- Purpura fulminans neonatalis
- Thrombophilia
- Venous thrombosis
- Von willebrand disease
- VWF gene
ASJC Scopus subject areas
- Medicine(all)