Alterations in Hemostasis
Chapter 22: Alterations in Hemostasis
Definition of Hemostasis
Hemostasis: Defined as "stoppage of blood flow."
Abnormal Hemostasis can include:
Inappropriate Clotting: Formation of clots when not needed.
Insufficient Clotting: Failure to form clots leading to excessive bleeding.
Key Points:
The liver synthesizes coagulation factors, which are crucial for hemostasis.
Vitamin K is essential for the formation of several clotting factors.
Hemostatic Response to Injury
Initial Response:
When injury occurs, there is a rapid vessel spasm due to:
Smooth muscle contraction of the vessel wall following endothelium injury.
This reaction serves to minimize blood loss immediately post-injury.
Formation of the Platelet Plug:
Von Willebrand factor (vWf) causes platelets to become 'sticky’, promoting aggregation and forming a platelet plug.
Coagulation Pathways
Intrinsic Pathway: Activated by damage to the endothelium.
Extrinsic Pathway: Activated by tissue injury.
Common Features in Both Pathways:
Both pathways lead to:
Activation of Factor Xa which converts prothrombin into thrombin.
Thrombin then converts fibrinogen into fibrin, forming a fibrin mesh that stabilizes the platelet plug.
Clot Retraction and Dissolution
Clot Retraction: The process by which the clot contracts and stabilizes the wound site.
Clot Dissolution (Lysis): The process involving plasminogen activators that convert plasminogen to plasmin, which dissolves the fibrin mesh. This mechanism is vital for healing and restoring normal blood flow.
Disorders of Hemostasis
Hypercoagulability
Causes of hypercoagulability include:
Increased platelet count (Thrombocytosis).
Symptoms that may arise:
Formation of thrombus (blood clots).
Development of embolus (clots that travel and obstruct vessels).
Virchow’s Triad
Virchow’s Triad refers to three factors that increase the risk of thrombosis:
Stasis: Immobility or pooling of blood.
Endothelial Injury: Occurs from surgery or trauma to blood vessels.
Hypercoagulable State: Influenced by smoking, genetics, birth control use, obesity, medications, etc.
Under Coagulation and Bleeding Disorders
Thrombocytopenia: Defined as a decreased number of platelets (<100,000).
Causes:
Decreased production due to bone marrow dysfunction.
Increased destruction caused by autoimmune disorders.
Symptoms can include excessive bleeding, purpura, ecchymosis (bruising), epistaxis (nosebleeds).
Diagnosis: Based on complete blood count (CBC), prothrombin time (PT), partial thromboplastin time (PTT).
Idiopathic Thrombocytopenic Purpura (ITP)
Characteristics:
Excessive destruction of platelets primarily in the spleen and liver.
More common in children following viral infections and in adults without an identifiable trigger.
Symptoms include purpura, petechiae, and bruising.
Treatment options include corticosteroids, immunoglobulins, or splenectomy for severe cases.
Impaired Platelet Function
Conditions that can cause impaired function include:
Kidney disease (renal failure).
Drugs (e.g., NSAIDs, aspirin, heparin).
Conditions leading to acidosis affecting functionality.
Bleeding Associated with Coagulation Factors
Coagulation factors include Factors V, VII, prothrombin, and fibrinogen, which are affected by:
Liver disease (disrupting factor synthesis).
Vitamin K deficiency, which is critical for synthesis of various clotting factors.
Von Willebrand Disease
Characterized by:
A defect in the Von Willebrand factor, critical for platelet adhesion.
Inherited in an autosomal pattern.
Symptoms include abnormal bleeding tendencies, especially during surgeries.
Treatment includes administration of factor VIII.
Hemophilia
A genetic disorder characterized by deficiencies in coagulation factors, primarily Factor VIII (Hemophilia A) or Factor IX (Hemophilia B).
It is X-linked recessive, predominantly affecting males, although 30% of cases may occur without a family history.
Symptoms include bleeding into joints (hemarthrosis), gastrointestinal tract, and soft tissues.
Treatment involves factor infusions and education on managing bleeding episodes.
Disseminated Intravascular Coagulation (DIC)
A complex disorder that is a secondary complication due to various clinical conditions (e.g., sepsis, trauma).
Features include:
Massive activation of the clotting cascade.
Formation of widespread fibrin clots leading to occlusive phenomena, tissue ischemia, and systemic hemorrhage.
Symptoms can vary based on the underlying cause.
Treatment includes relieving the underlying condition and supporting clotting factor replacement (e.g., transfusions).
Potential risks include profound bleeding due to depleted clotting factors.