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  • imageHemophilia is an inherited bleeding disorder resulting from a congenital deficiency in factor VIII or IX.

  • imageThe goal of therapy for hemophilia is to prevent bleeding episodes and their resulting long-term complications, and to arrest bleeding if it occurs.

  • imageRecombinant factor concentrates usually are the first-line treatment of hemophilia because they have the lowest risk of infection.

  • imageInhibitor formation is the most significant treatment complication in hemophilia and is associated with significant morbidity and decreased quality of life.

  • imageRecombinant factor VIIa is effective for the treatment of acute bleeds in patients with hemophilia A or B who have developed inhibitors.

  • imageThe goal of therapy for von Willebrand disease (vWD) is to increase von Willebrand factor (vWF) and factor VIII levels to prevent bleeding during surgery or arrest bleeding when it occurs.

  • imageFactor VIII concentrates that contain vWF are the agents of choice for treatment of type 3 vWD and some type 2 vWD, and for serious bleeding in type 1 vWD.

  • imageDesmopressin acetate often is effective for the treatment of type 1 vWD. It also may be effective for the treatment of some forms of type 2 vWD in addition to mild-to-moderate hemophilia A.


Patient Care Process for Coagulation Disorders



  • Patient characteristics (age, sex, etc.)

  • Patient medical history (including family history and bleeding history)

  • Patient surgical history (including dental procedures)

  • Social history (level of activity, extracurricular activities)

  • Medication list, especially aspirin, NSAIDs, other antiplatelet medications and anticoagulants

  • Objective data

    • Vital signs (blood pressure, heart rate, respiratory rate, etc.)

    • Labs vary dependent on likely cause: prothrombin time (PT), activated partial thromboplastin time (aPTT), platelets, hemoglobin (Hgb), factor VIII level, factor IX level, von Willebrand factor antigen and activity


  • Presence of active bleeding (see section “Clinical Manifestations and Diagnosis” and Table 119-1)

  • Presence of common clinical manifestations in bleeding disorders (easy bruising, bleeding after surgery, mucocutaneous bleeding, prolonged menses, etc. and Table 119-1).

  • Factor level and the need for prophylactic management.


  • Determine drug therapy regimen (dose, route, frequency, and duration)

    • Appropriate factor therapy (Table 119-2, 119-3)

    • Rescue and/or as-needed therapy (factor, desmopressin, aminocaproic acid, tranexamic acid, etc.)

  • Monitoring parameters (signs/symptoms of bleeding, frequency of bleeding episodes)

  • Patient education/counseling (control of bleeding episodes, administration of drug therapy, when to seek medical attention)

  • Continued care at a Hemophilia Treatment Center (or comparable clinic with a comprehensive care team)


  • Provide education and reinforcement of the treatment plan

  • Improve adherence through motivational interviewing and open discussions about patient care and the treatment plan

  • Schedule regular clinic appointments to assess adherence, bleeding episodes, and to tailor patient therapy

Follow-up: Monitor and Evaluate

  • Control and resolution of bleeding signs and symptoms

  • Number of bleeding episodes (improvement since beginning care, need for adjustment in the treatment plan)

  • Factor level activity (tailor factor doses to patient-specific levels)

  • Adherence to the treatment plan (prophylactic and as-needed therapy, seeking ...

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