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Condition/Disorder Synonyms

  • Coagulation defects

  • Disorders of blood coagulation

ICD-9-CM Codes

  • 286.5 Hemorrhagic disorder due to intrinsic circulating anticoagulants

  • 286.52 Acquired hemophilia

  • 286.53 Antiphospolipid antibody with hemorrhagic disorder

  • 286.59 Other hemorrhagic disorder due to intrinsic circulating anticoagulants, antibodies, or inhibitors

ICD-10-CM Codes

  • D68.311 Acquired hemophilia

  • D68.312 Antiphospholipid antibody with hemorrhagic disorder

  • D68.318 Other hemorrhagic disorder due to intrinsic circulating anticoagulants, antibodies, or inhibitors

Preferred Practice Patterns

Key Features


  • Disorders involving slower than normal blood clotting

  • Occurs spontaneously or as an excessive response to injury leading to bleeding into joints (termed hemarthrosis) or tissues

Essentials of Diagnosis

  • Classification

    • - Hepatic disease

    • - Vitamin K deficiency

    • - Renal disease

  • Laboratory values

  • Identifying the underlying cause

General Considerations

  • Vitamin K deficiency can be a result of malabsorption disorders

  • Liver disorders

    • - Decreased synthesis of coagulation

    • - Impaired clearance of activated haemostatic components


  • Acquired or hereditary

  • Factor V Leiden mutation in 5% of caucasian population

  • Vitamin K deficiency can occur in a newborn or later stages in life where there is intestinal malabsorbtion5

Clinical Findings

Signs and Symptoms

  • Shock including end organ dysfunction3

  • Diffuse bleeding

    • - Hematuria

    • - Melena

    • - Purpura

    • - Petechiae

  • Thrombotic lesions

    • - Major vessel thrombosis

    • - Purpura fulminans

Functional Implications

  • Bleeding episodes

  • Excessive bruising

Possible Contributing Causes

  • Hemophilia3

  • Endothelial damage from endotoxin or virus

  • Tissue necrosis from burns

  • Diffuse ischemic injury from shock or hypoxia acidosis

  • Systemic release of tissue procoagulants from cancer and placental disorders

  • Liver disease

  • Vitamin K deficiency

  • Von Willebrand disease

  • Broad spectrum antibiotics

  • Fulminant hepatitis

  • Acute fatty liver from pregnancy

Differential Diagnosis

  • Coagulopathy of liver disease (hepatic synthetic dysfunction)

Means of Confirmation or Diagnosis

Laboratory Tests

  • CBC

  • Coagulation tests

  • Platelet count

  • Clotting time

  • Bleeding time (BT)

  • Prothrombin time (PT)

  • Partial thromboplastin time (PPT)

  • Thrombin time (TT)

Findings and Interpretation

  • Consumptive coagulopathy3

    • - Prolonged

      • aPTT,

      • Prothrombin time (PT)

      • Thrombin time

  • Increase in fibrin-fibrinogen split products (FSP)

  • Elevated levels of plasma D-dimer

  • Decreased plasma fibrinogen or platelets



  • Replacement therapy for concumptive coagulopathy

  • Anticoagulant therapy for coagulation activation

  • Specific factor concentrates


  • Genetic counselor

  • Physican for laboratory tests



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