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IV immunoglobulin and anti RH Dwhen unresponsive to corticosteroids or spleenectomy or spleenectomy isn’t an option.Rituximab to lyse B cell and reduce immune recognition of platelets.Effectiveness of spleenectomy is based on 4 factors.Thrombopoietin receptor agonist to increase platelet production.Platelet transfusion may cause further antibody formation.Anti-fibrinolytic agent may be used for severe bleeding. Treatment Specific: Thrombotic thrombocytopenic purpuraPlasmapheresis is used to aggressively reverse platelet consumption by supplying the 10
appropriate vWf and enzyme and removing large vWf molecules that bind with platelets.CorticosteroidsSpleenectomyPlatelet transfusion generally is contraindicated because this may lead to new vWf platelet complex and increased clotting. Treatment Specific: Heparin Induced Thrombocytopenia Discontinue heparin and start direct thrombin inhibitor.Warfarin sould be started when platelet is at least 150,000.If clotting is severe, Plasmapheresis to clear the plateletaggregating igG from blood, protamine sulfate, thrombolytic agents, or surgery to remove clots.Platelet transfusion is NOT effective because it enhance thromboembolic events.Never give heparin or LMWH.Treatment Specific: Acquired thrombocytopenia from Decreased Platelet ProductionIf the precipitating factor is unknown, the patient may receive corticosteroid.Platelet transfusion is given if life-threatening hemorrhage develops. Hemophilia: X-linked recessive disorder caused by defective coagulation factor 8 or 9.Hemophilia AFactor 8 deficiency, recessive, transmitted by female carriers, displayed almost exclusively in men.Most common hemophilia.Hemophilia BFactor 9 (Christmas disease) deficiency, recessive, transmitted by female, displayed almost exclusively in menVon Willebrand DiseaseAutosomal dominant, seen in both genders. (Recessive in severe form).Deficiency of von Willebrand coagulation protein.Factor 8 is bind with von WillebrandMost common congenital bleedingdisorder.TreatmentPrevent and treat bleedingReplacement therapy may be given before surgery and dental care.Desmopressin to stimulate an increase in factor 8 and VWF. Thisdrug act on platelets and endothelial cells to cause the release of VWF which bind with factor8, thus increasing their concentration. IV/INAnti-fibrinolytic therapy inhibits fibrin Lysis by inhibiting plasminogen activation in the fibrin clot, thereby enhancing clot stability.ComplicationsDevelopment of antibodies/inhibitor for factor 8 or 9.Transfusion complication.Thrombotic episode with use of factor 9.