RT Book, Section A1 Diaz, Rosa A1 Yee, Donald L. A2 Kline, Mark W. SR Print(0) ID 1182910120 T1 Inherited Bleeding Disorders T2 Rudolph's Pediatrics, 23e YR 2018 FD 2018 PB McGraw-Hill Education PP New York, NY SN 9781259588594 LK accesspediatrics.mhmedical.com/content.aspx?aid=1182910120 RD 2024/03/28 AB Inherited bleeding disorders are a heterogeneous group of conditions that include disorders of primary and secondary hemostasis. During primary hemostasis, platelets adhere and aggregate at the site of vascular injury to form a platelet plug. Bleeding into the skin (eg, petechiae and/or ecchymosis) and mucous membranes typifies disorders of primary hemostasis such as von Willebrand disease (VWD). Secondary hemostasis leads to stabilization of the platelet plug through activation of the coagulation cascade and production of cross-linked fibrin. Patients affected by a disorder of secondary hemostasis such as hemophilia or 1 of the rare bleeding disorders (RBDs) classically bleed into soft tissues, muscles, and joints. In general, children with a bleeding disorder may present with severe or life-threatening bleeding episodes early in life, or in a more indolent manner, with symptoms that may not seem very unusual (eg, frequent nosebleeds or heavy menstrual bleeding). Recognizing the clinical and laboratory features that suggest the presence of an inherited bleeding disorder is paramount to optimizing treatment and long-term outcomes.