Bleeding & Coagulation Disorders

• Hemophilia A is the most common type of hemophilia, affecting males more than females due to its x-linked recessive disorder.
• Hemophilia A is characterized by a deficiency in factor VIII, also known as factor A.
• Hemorrhoids are a unique clinical manifestation of Hemophilia A, representing close to 80 percent of hemorrhages in ambulatory patients with hemophilia.
• Hemophilia B is a deficiency in clotting factor IX, also known as factor IX or B9.
• Warfarin-induced skin necrosis is a condition that occurs when a patient begins to develop necrotic lesions on their legs and feet due to a deficiency in vitamin K-dependent plasma protein, likely protein C.
• The reason for bridging with heparin or low molecular weight heparin in high-risk patients is due to the transient prothrombotic state caused by protein C.
• Clinical manifestations of VWD include muco cutaneous bleeding, excessive bleeding, and increased susceptibility to bruising.
• Testing for VWD can include a von Willebrand factor antigen or activity test, which may show decreased or no platelet aggregation.
• Von Willebrand Disease is a problem with platelet adhesion, which can be exacerbated by the use of aspirin and other antiplatelet medications.
• Von Willebrand Factor (VWF) binds to platelets, forming a platelet plug that stops bleeding.
• There are three subtypes of VWD: type 1, type 2, and type 3.
• Von Willebrand Disease (VWD) is the most common inherited bleeding disorder, affecting up to 1% of the population.
• Diagnosis of VWD involves a normal PT but prolonged PTT due to decreased factor VIII activity.
• In factor five leiden, the point mutation prevents protein c from inactivating factor five, leading to excessive clotting.
• Aristocetin, when added to the plasma of a patient with von Willebrand Disease, does not cause platelet aggregation, indicating that the patient likely has von Willebrand Disease.
• Desmopressin, also known as DDAVP, causes the release of endogenous von Willebrand Factor and is the main treatment for von Willebrand Disease.
• Von Willebrand Factor Concentrates are used in patients with severe von Willebrand Disease or Type 3 Disease, as they have little to no endogenous von Willebrand Factor.
• Factor Five Leiden is the most common cause of inherited hypercoagulable state.
• Factor Five Leiden is a single point mutation in factor five which leads to a hypercoagulable state.
• Factor Five Leiden causes factor five to no longer be able to be cleaved, a process that is used to inactivate factor five.
• Protein C, a protein that comes from the liver, is responsible for inactivating factor five.
• Another treatment option for hemophilia A is desmopressin, also known as DDAVP, which stimulates the release of von Willebrand factor.
• Hemophilia A is characterized by factor VIII deficiency, which can be confirmed by a factor VIII assay.
• Von Willebrand factor partners with factor VIII and binds to and stabilizes factor VIII, increasing its stability.
• PTT measures the intrinsic and common coagulation pathway, including factors 8, 9, 11, and 12.
• The kid may complain of persistently painful joints, indicating hemarthrosis.
• Hemophilia B is characterized by factor IX deficiency, which can be confirmed by a factor IX assay.
• Von Willebrand factor is not involved in factor IX deficiency, which is why it is not an option for hemophilia B.
• The patient's prothrombin is normal, but their partial thromboplastin time (PTT) is prolonged.
• Treatment for hemophilia A includes factor VIII infusions, which can be used for prophylaxis or during an acute lead.
• In Von Willebrand Disease, there is a decrease in the quality or quantity of von Willebrand factor, leading to ineffective platelet adhesion and bleeding.
• Hemophilia A presents with a male patient, x-linked recessive disorder, increased PTT due to what it measures, and can use factor eight and DDAVP.
• Von Willebrand factor's job is to act like glue for platelets, sticking them together to form a platelet plug to close up an injury to a blood vessel.
• Hemophilia B is almost identical to Hemophilia A, with two differences: it is a factor 9 deficiency, not factor 8, and DDAVP is not used as it is specific to factor eight.
• In Hemophilia B, 80% of patients are diagnosed with hemorrhoids.
• Von Willebrand Disease is a decrease in the quality or quantity of von Willebrand factor which leads to bleeding.
• The clinical manifestations of Hemophilia B are identical to Hemophilia A.
• Treatment for Hemophilia B includes factor 9 infusions for prophylaxis during acupully, but DDAVP is not used as it is specific to factor eight.
• In diagnosis, a factor 9 assay can be checked to see if it's decreased or absent.
• DDAVP is an option for Hemophilia A, but not for Hemophilia B as it is a factor 9 deficiency, not factor 8.