Podcast 529: Hemophilia

<p><strong>Contributor: Aaron Lessen, MD</strong></p> <p><strong>Educational Pearls:</strong></p> <ul> <li style="font-weight: 400;"><span style= "font-weight: 400;">Hemophilia is characterized by bleeding (A /B) A is is 8, B (chrstmas, 9) </span></li> <li style="font-weight: 400;"><span style= "font-weight: 400;">Hemophilia refers to a group of bleeding disorders caused by a deficiency in a certain clotting factor. The two most common are hemophilia A (caused by a lack of factor VIII), and B (caused by a lack of factor 9)</span></li> <li style="font-weight: 400;"><span style="font-weight: 400;">Most cases are inherited in an X-linked recessive manner. Therefore, males are the most commonly affected. However, spontaneous cases do occur in all sexes.</span></li> <li style="font-weight: 400;"><span style= "font-weight: 400;">People with hemophilia can present to the ED with a variety of bleeding complications including hemarthrosis, intracranial hemorrhage, and GI bleeds. </span></li> <li style="font-weight: 400;"><span style= "font-weight: 400;">Treatment is guided by the type of hemophilia and the degree of bleeding, with the most severe cases being treated with replacement of 100% of that person’s deficient clotting factor. </span></li> <li style="font-weight: 400;"><span style= "font-weight: 400;">Timely treatment improves outcomes, so consider empiric therapy in those with known hemophilia</span></li> </ul> <p><strong>References</strong></p> <p><span style="font-weight: 400;">Hemophilia A in the third millennium.Franchini M, Mannucci PM Blood Rev. 2013 Jul;27(4):179-84. Epub 2013 Jun 28. </span></p> <p><span style="font-weight: 400;">Rheumatic manifestations of hematologic disorders.Aviña-Zubieta JA, Galindo-Rodriguez G, Lavalle C Curr Opin Rheumatol. 1998;10(1):86. </span></p> <p><span style="font-weight: 400;">The molecular genetics of hemophilia: blood clotting factors VIII and IX.Lawn RM Cell. 1985;42(2):405. </span></p> <p><span style="font-weight: 400;">Guidelines for the management of hemophilia.Srivast