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Condition/Disorder Synonyms

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  • Coagulation defects

  • Disorders of blood coagulation

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ICD-9-CM Codes

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  • 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

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ICD-10-CM Codes

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  • D68.311 Acquired hemophilia

  • D68.312 Antiphospholipid antibody with hemorrhagic disorder

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

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Preferred Practice Patterns

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Key Features

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Description

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  • 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

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Essentials of Diagnosis

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  • Classification

    • - Hepatic disease

    • - Vitamin K deficiency

    • - Renal disease

  • Laboratory values

  • Identifying the underlying cause

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General Considerations

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  • Vitamin K deficiency can be a result of malabsorption disorders

  • Liver disorders

    • - Decreased synthesis of coagulation

    • - Impaired clearance of activated haemostatic components

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Demographics

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  • 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

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Clinical Findings

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Signs and Symptoms

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  • Shock including end organ dysfunction3

  • Diffuse bleeding

    • - Hematuria

    • - Melena

    • - Purpura

    • - Petechiae

  • Thrombotic lesions

    • - Major vessel thrombosis

    • - Purpura fulminans

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Functional Implications

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  • Bleeding episodes

  • Excessive bruising

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Possible Contributing Causes

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  • 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

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Differential Diagnosis

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  • Coagulopathy of liver disease (hepatic synthetic dysfunction)

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Means of Confirmation or Diagnosis

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Laboratory Tests

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  • CBC

  • Coagulation tests

  • Platelet count

  • Clotting time

  • Bleeding time (BT)

  • Prothrombin time (PT)

  • Partial thromboplastin time (PPT)

  • Thrombin time (TT)

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Findings and Interpretation

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  • 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

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Treatment

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Medications

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  • Replacement therapy for concumptive coagulopathy

  • Anticoagulant therapy for coagulation activation

  • Specific factor concentrates

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Referrals/Admittance

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  • Genetic counselor

  • Physican for laboratory tests

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Impairments

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