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Harrison's Principles of Internal Medicine, 18e
| Part 7. Oncology and Hematology > Section 3. Disorders of Hemostasis > |
Chapter 116. Coagulation Disorders
Sections: Coagulation Disorders: Introduction, Hemophilia, Rare Bleeding Disorders, Further Readings.
Topics Discussed: blood coagulation tests; pathophysiology of blood disorders.
Excerpt:"Deficiencies of coagulation factors have been recognized for centuries. Patients with genetic deficiencies of plasma coagulation factors exhibit life-long recurrent bleeding episodes into joints, muscles, and closed spaces, either spontaneously or following an injury. The most common inherited factor deficiencies are the hemophilias, X-linked diseases caused by deficiency of factor (F) VIII (hemophilia A) or factor IX (FIX, hemophilia B). Rare congenital bleeding disorders due to deficiencies of other factors, including FII (prothrombin), FV, FVII, FX, FXI, FXIII, and fibrinogen are commonly inherited in an autosomal recessive manner (Table 116–1). Advances in characterization of the molecular bases of clotting factor deficiencies have contributed to better understanding of the disease phenotypes and may eventually allow more targeted therapeutic approaches through the development of small molecules, recombinant proteins, or cell and gene-based therapies...."
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