Medical Importance
Patients with a wide variety of inherited and acquired bleeding
disorders are encountered in nearly all subspecialties of medicine.
Most of these disorders can now be identified with appropriate
laboratory testing. Screening tests can rapidly distinguish platelet
function defects, coagulation disorders and forms of excessive
(systemic) fibrinolysis. Assays are also available for most of the
components in these pathways, allowing identification of the specific
defect in hemostasis. In many cases, the pathogenesis is now understood
on a molecular level. With inherited hemorrhagic disorders, accurate
diagnosis and understanding of the inheritance is necessary for genetic
counseling and identification of family members at risk. Finally,
specific therapy is now available for both prophylaxis and treatment of
bleeding due to several of these disorders.
Objectives
- Be able to briefly describe the pathogenesis, diagnostic
tests, and therapeutic approach to patients with the following acquired
disorders who are actively bleeding:
- end stage liver disease
- acquired factor VIII inhibitor (auto‑antibody against FVIII)
- severe DIC due to acute promyelocytic leukemia
- vitamin K deficiency
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