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  • Hemophilia A
  • Classic hemophilia
  • Hemophilia B
  • Christmas disease
  • Coagulation defects
  • Disorders of blood coagulation

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  • 286.0 Congenital factor VIII disorder
  • 286.1 Congenital factor IX disorder
  • 286.2 Congenital factor XI deficiency
  • 286.3 Congenital deficiency of other clotting factors
  • 286.4 Von Willebrand’s disease

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  • D66 Hereditary factor VIII deficiency
  • D67 Hereditary factor IX deficiency
  • D68.0 Von Willebrand’s disease
  • D68.1 Hereditary factor XI deficiency
  • D68.2 Hereditary deficiency of other clotting factors

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Description

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  • Disorders involving slower than normal blood clotting that occurs spontaneously or as an excessive response to injury, leading to bleeding into joints (hemarthrosis) or tissues

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Essentials of Diagnosis

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General Considerations

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  • Genetic factor deficiencies occur almost exclusively in males with females as asymptomatic carriers
  • Hemophilia classified as mild (asymptomatic), moderate (excessive bleeding after minor injury,) or severe
  • Bleeding into joint results in fibrosis
  • Von Willebrand’s disease results in bleeding into skin or mucous membranes rather than joints or muscles

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Demographics

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  • Inherited
  • Factor VIII deficiency occurs in 1 of every 5,000-10,000 males
  • Factor IX deficiency occurs in 1 of every 50,000 people2
  • Von Willebrand’s disease most common bleeding disorder in Caucasians4
  • Von Willebrand’s disease seen in both males and females7

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Signs and Symptoms

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  • Pain
  • Erythema
  • Edema
  • Bruises easily
  • Bleeding into joints or muscles without history of injury
  • Excessive bleeding that results from injury

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Functional Implications

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  • Joint contractures
  • Hematoma of muscles can cause compartment syndrome3

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Possible Contributing Causes

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  • Genetic x-linked recessive (such as factor VIII, or IX deficiencies) or autosomal dominant disorder (such as Von Willebrand’s disease)
  • Multiple episodes of bleeding in same joint can cause hemophilic arthritis (hemarthropathy) 7

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Differential Diagnosis

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  • Platelet disorder

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Laboratory Tests

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  • Platelet count
  • Clotting time
  • Bleeding time
  • Prothrombin time
  • Partial thromboplastin time
  • Thrombin time
  • DNA analysis
  • Factor assays
  • Fibrinogen test

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Imaging

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  • Standard radiograph for hemarthropathy
  • Magnetic resonance imaging (MRI) or computerized axial tomography (CT scan) for head trauma or organ involvement

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  • Factor VIII deficiency: prolonged partial thromboplastin time, normal prothrombin and bleeding times
  • Von Willebrand’s disease: prolonged partial thromboplastin and bleeding times, normal prothrombin time

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Medications

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  • Injection or infusion of blood concentrate of the deficient factor
  • Desmopressin acetate for Von Willebrand’s disease4 or factor VIII deficiency5
  • Amicar

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