Coagulation Disorders

What Are Coagulation Disorders?

Coagulation disorders are conditions that affect the body’s ability to form blood clots. Some disorders increase the risk of spontaneous bleeding, while others increase the risk of forming clots. Clotting disorders can affect any of the steps in the production of clots from the coagulation cascade. They are caused by genetic or acquired diseases or conditions, as well as by certain medications.

Types of Coagulation Disorders

Coagulation disorders can be divided into two types, hypercoagulable (thrombophilic) states and hypocoagulable (bleeding) disorders. The two types of disorders can occur together, and their effects on clotting may change over time.

Hypercoagulable (Thrombophilic) States

Hypercoagulable (thrombophilic) states are conditions that increase the risk of forming clots. They can be caused by genetic or acquired diseases or conditions, as well as by certain medications. Examples include:

  • Factor V Leiden thrombophilia
  • Antithrombin deficiency
  • Antiphospholipid antibody syndrome
  • Protein C deficiency
  • Protein S deficiency
  • Elevated factor VIII levels
  • Elevated homocysteine levels
  • Factor XIII deficiency
  • Paroxysmal nocturnal hemoglobinuria

Hypocoagulable (Bleeding) Disorders

Hypocoagulable (bleeding) disorders are conditions that interfere with the ability of the blood to form clots. This leads to an increased risk of spontaneous bleeding. Examples include:

  • Von Willebrand Disease (VWD)
  • Hemophilia A and B
  • Deficiencies in clotting factors (i.e. factor IX, X, XI, XII)
  • Platelet disorders

Diagnosing Coagulation Disorders

Diagnosing a coagulation disorder typically requires laboratory testing. The specific tests used will depend on the type of disorder suspected. Tests may include:

  • Red blood cell count
  • White blood cell count
  • Platelet count
  • Prothrombin time (PT)
  • Activated partial thromboplastin time (aPTT)
  • Fibrinogen
  • D-dimer
  • Thrombin time
  • Factor V Leiden mutation
  • Antithrombin III deficiency
  • Antiphospholipid antibody testing
  • Protein C and S testing
  • Homocysteine level
  • Factor XIII activity
  • Paroxysmal nocturnal hemoglobinuria screening
  • Factor VIII level
  • Von Willebrand factor level
  • Hemophilia A and B levels

Treatment of Coagulation Disorders

Treatment of coagulation disorders depends on the type of disorder. Hypercoagulable states can usually be managed with lifestyle modifications, such as stopping certain medications, avoiding long periods of sitting or standing, and wearing support stockings. It may also involve anticoagulation therapy or surgical interventions. For hypocoagulable states, treatment may involve the use of blood clotting medications, such as factor concentrates or antifibrinolytic agents. Surgery or other interventions may also be required for some conditions.