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vonWillebrands Disease

vonWillebrands Disease . Dr. Erik VonWillebrand . In 1925, Dr. vonWillebrand evaluated a family for excessive bleeding In 1928, after more research, he did not believe the platelets were involved in the cause for the bleeding and he named this newly discovered disease “pseudohemophilia”

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vonWillebrands Disease

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  1. vonWillebrands Disease

  2. Dr. Erik VonWillebrand • In 1925, Dr. vonWillebrand evaluated a family for excessive bleeding • In 1928, after more research, he did not believe the platelets were involved in the cause for the bleeding and he named this newly discovered disease “pseudohemophilia” • The disease went undiagnosed until the 1970’s when the actual cause was identified and named after the Finnish doctor

  3. Signs of vonWillebrands Disease • Easy Bruising • Frequent or prolonged nosebleeds • Heavy or prolonged menstrual periods • Prolonged bleeding after surgery or dental work

  4. At the Site of Injury Blood has four major components 1. plasma 2. red blood cells 3. white blood cells 4. platelets

  5. Primary Hemostasis Step 1 Vasoconstriction, the creation of a clot. Platelets rush to the injury site Step 2 Platelet Plug Formation and stabilization vonWillebrands is the platelet glue, and when absent, the clot breaks loose, it does not cause the bleed, but bleeding will be prolonged

  6. Differences between hemophilia and vWD • 1 in 5,000 males • Sex linked, recessive • Genetic • Joint and muscle bleeds • Usually severe • Fibrin clot • Requires intravenous clotting factor • 1 in 50 people • Autosomal • Genetic or acquired • Mucocutaneous bleeds • Most cases are mild • Platelet plug • Oral meds and nasal sprays, etc • Acts like a bodyguard for Factor VIII • Type O is most prevalent Hemophilia vonWillebrands

  7. 3 Main Types of vonWillebrands Type 1 Quantitative Disorder • Low levels of vonWillebrands • Most common • Type 2 Qualitative Disorder • Four disorders fall under this type • All four have to do with qualitative abnormal functions • Type 3 • Lack of vonWillebrand Factor • and low Factor VIII

  8. Diagnosis of vWD in the Lab • Bleeding time • CBC Count • Platelet Count • PT/PTT • VWF antigen & Factor VIII • Ristocetin Co-Factor • RIPA These tests will not diagnose vWD, must order secondary tests

  9. Elements that Influence Coagulation Testing Elements that Raise Levels • Estrogen/Progesterone • Adrenaline • Recent transfusion • Hyper Thyroidism • Diabetes • Malignancy • Age Elements that lower Levels • Blood Type O • Hypo Thyroidism

  10. Treatments of vWD • Amicar • prevents re-bleeding • Keeps clot in place • Comes in syrup or tablets • DDAVP (desmopressin acetate) • Treatment for Type 1 and 2 • Spray • Injection • Humate P & Wilate • Treatment for Type 3 • Contains both vWF and Factor VIII • Only two that are FDA approved

  11. Remember the Four Warning Signsof vonWillebrands Disease • Easy Bruising • Frequent or Prolonged Nose Bleeds • Heavy or Prolonged Menstrual Bleeding • Prolonged bleeding after surgery, childbirth, trauma or invasive dental work

  12. For More Information Utah Hemophilia Foundation Address: 772 E 3300 S Suite 210 Salt Lake City UT 84106 Phone: (801) 484-0325 Fax: (801) 746-2488 or Contact Your Local Axiom Therapeutics Representativeat Axiomtherapeutics.com

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