{"id":9564,"date":"2014-03-10T18:28:00","date_gmt":"2014-03-10T22:28:00","guid":{"rendered":"https:\/\/crashingpatient.com\/?p=9564"},"modified":"2014-03-10T18:28:00","modified_gmt":"2014-03-10T22:28:00","slug":"hemophilia-von-willebrands-disease","status":"publish","type":"post","link":"https:\/\/crashingpatient.com\/medical-surgical\/hematology\/hemophilia-von-willebrands-disease.htm\/","title":{"rendered":"Hemophilia and von Willebrand’s Disease"},"content":{"rendered":"

<\/span>Hemophilia A<\/span><\/h2>\n

x linked recessive causing defective VIII:c,<\/p>\n

Abnormal ptt.<\/p>\n

Replace c cryoprecipitate of factor VIII 1 unit=100 units VIII<\/p>\n

1 cc of FFP=1 unit VIII and less of IX<\/p>\n

dDAVP raises factor VIII by causing release from endothelial cells, It has ADH-like effects.\u00a0 Dose:\u00a0 0.3 mcg\/kg.<\/p>\n

Number of units=0.5 x (Desired Factor Level) x (Weight in Kg)\u00a0\u00a0\u00a0\u00a0\u00a0 (1\/2 life of 12 hours)\u00a0 (Each unit of factor\/kg raises levels by 2%)<\/p>\n

Raise to 100% in head injury, mult. Trauma, neck\/tongue\/throat hematoma, ABD pn\/GI bleed, eye trauma.<\/p>\n

VIII-plasma or recombinant<\/p>\n

 <\/p>\n

L<\/strong><\/p>\n

evels for Hem A\/B<\/p>\n

    \n
  • Joint\/Tissue-50% A, 30% B impending joint will present with bubbly feeling, tingling, warmth<\/li>\n
  • Mouth-correct 50\/30%,\u00a0\u00a0 Amicor 100 mg\/kg (max 24 g\/day) Q6 x 1 week<\/li>\n
  • Epistaxis-local measures the factor 50\/30<\/li>\n
  • Iliopsoas hemorrhage-100\/80% then maintain above 40<\/li>\n
  • Muscle-50\/30<\/li>\n
  • Neck\/Retropharyngeal-100\/80<\/li>\n
  • Urinary-vigorous hydration, if still bleeding, replace factor.\u00a0 Prednisone 2mg\/kg divided TID x 2 days<\/li>\n
  • Suturing-replace factor first<\/li>\n
  • Trauma-100% replacement<\/li>\n
  • Fractures-100\/80 prior to splinting<\/li>\n
  • Abd Pain-100\/80<\/li>\n<\/ul>\n

    10 minute post transfusion, get PTT and factor level<\/p>\n

     <\/p>\n

    <\/span>Hemophilia B (Christmas)<\/span><\/h2>\n

    Factor IX, replace c factor<\/p>\n

    1 x Factor Level x Kg (1\/2 life of 24 hours)<\/p>\n

     <\/p>\n

    (Benefix is only 80% factor IX so multiply by 1.25 instead of 1)<\/p>\n

     <\/p>\n

    IX-older plasma derived can be thrombogenic, use recombinant<\/p>\n

    <\/span>von Willebrand\u2019s Disease<\/span><\/h2>\n

    Autosomal (usually dominant)<\/p>\n

    Presents with mucocutaneous\u00a0 bleeding, commonly epistaxis, gi bleeds, and prolonged menorrhagia.<\/p>\n

    Patients will have prolonged PTT, increased bleeding time, Normal PT and Platelet count.<\/p>\n

    1 bag cryo per 10 kg or FFP in emergency<\/p>\n

    Consider if prolonged vaginal bleeding in menarche or in women in the perimenopausal period.\u00a0 Estrogen and progesterone raise vWF level.\u00a0 When Anovulatory cycles are present, may see prolonged bleeding.<\/p>\n

    Treat with dDAVP, Factor VIII concentrates with high vWF levels (Humate-P or Koate-HS), Cryoprecipitate (10 units\/kg), FFP<\/p>\n","protected":false},"excerpt":{"rendered":"

    Hemophilia A x linked recessive causing defective VIII:c, Abnormal ptt. Replace c cryoprecipitate of factor VIII 1 unit=100 units VIII 1 cc of FFP=1 unit VIII and less of IX dDAVP raises factor VIII by causing release from endothelial cells, It has ADH-like effects.\u00a0 Dose:\u00a0 0.3 mcg\/kg. Number of units=0.5 x (Desired Factor Level) x […]<\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_genesis_hide_title":false,"_genesis_hide_breadcrumbs":false,"_genesis_hide_singular_image":false,"_genesis_hide_footer_widgets":false,"_genesis_custom_body_class":"","_genesis_custom_post_class":"","_genesis_layout":"","footnotes":""},"categories":[25],"tags":[],"yoast_head":"\nHemophilia and von Willebrand's Disease - Crashing Patient<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/crashingpatient.com\/medical-surgical\/hematology\/hemophilia-von-willebrands-disease.htm\/\" \/>\n<meta name=\"twitter:label1\" content=\"Written by\" \/>\n\t<meta name=\"twitter:data1\" content=\"CrashMaster\" \/>\n\t<meta name=\"twitter:label2\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data2\" content=\"1 minute\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\/\/schema.org\",\"@graph\":[{\"@type\":\"WebPage\",\"@id\":\"https:\/\/crashingpatient.com\/medical-surgical\/hematology\/hemophilia-von-willebrands-disease.htm\/\",\"url\":\"https:\/\/crashingpatient.com\/medical-surgical\/hematology\/hemophilia-von-willebrands-disease.htm\/\",\"name\":\"Hemophilia and von Willebrand's Disease - 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