内科学-培训课件PPT
内科学-培训课件PPT,内科学,培训,课件,PPT
Hemorrhagic DisordersHuang Zhen-qian,ProfessorDept.of Hematology The First Affiliated Hospital,Guangzhou Medical U1ContentSummaryCauses and Mechanism of DiseaseCauses and Mechanism of DiseaseClinical FeaturesClinical FeaturesInvestigation of LabInvestigation of LabCriterion of DiagnosisCriterion of DiagnosisManagementManagement2DefinitionvWhen the normal hemostatic mechanism fails,major hemorrhage may follow minor trauma or may arise apparently spontaneously.3Mechanism of normal hemostasisVascular mechanismPlatelet mechanismBlood coagulation mechanism4Normal hemostasis567red thrombus8Anticoagulation and fibrinolystic mechanismvAnticoagulation systemAT-Protein C system:PC,PS,TMTFPIHeparin910Anticoagulation and fibrinolystic mechanismvFibrinolysis systemPlasminogenTissue-type plasminogen activator(t-PA)Urokinase-type plasminogen activator(u-PA)Inhibitors:2-plasmin inhibitor(2-PI),plasminogen activator inhibitor-1(PAI-1)11Anticoagulation and fibrinolystic mechanismPlasminogenActivatorInhibitorKallekrein (intrinsic)t-PA,u-PA PAI(extrinsic)2-PI plasmin fibrinogen(fibrin)FDP1213Classificationvvascular disordersvplatelet disordersvdisorders of blood coagulationvdisorders of anticoagulation system and fibrinolytic systemvComplex disorders of hemostatic mechanism14Classification:Vascular disordersvHereditary or congenital:Hereditary hemorrhagic telangiectasia(遗传性出血性毛细血管扩张症)遗传性出血性毛细血管扩张症),hereditary disorders of connective tissue such as Ehlers-Danlos syndrome.vSecondary:infections,chemical factors or drugs,disorders of metabolism(Vitamin C or P deficiency),pathological changes of vascular wall(atherosclerosis),connective tissue diseases.vAllergy:allergic purpura.vOther purpuras:purpura simplex,senile purpura,mechanic purpura,paraproteinemia.15Classification:platelet disordersThrombocytosisThrombocytopathyThrombocytopenia16Classification:platelet disorders17Classification:platelet disordersvThrombocytosisPrimary:essential thrombocythemiaSecondary:infections,injury,post-splenectomy,chronic myelocytic leukemia,other myeloproliferative disorders(such as polycythemia vera 真性红细胞增多症)真性红细胞增多症))18Classification:platelet disordersvFunctional abnormalities of plateletsHereditary:giant platelet syndrome(Bernard-Soulier syndrome),thrombasthenia(血小板无力症血小板无力症),Acquired:due to drugs,uremia,liver diseases,dysproteinemias.19Classification:disorders of blood coagulationvHereditary or congenital:hemophilia A(F deficiency),hemophilia B(F deficiency),factor deficiency(formerly hemophilia C),hypothrombinogenemia,hypofibrinogenemia,von Willebrands disease,other coagulation factors deficiency,including deficiency of activated-protein C inhibitor structural abnormalities.vAcquired:Vitamin K deficiency,severe liver diseases,disseminated intravascular coagulation(DIC)etc.20Classification:disorders of anticoagulation system and fibrinolytic systemvdisorders of anticoagulation system and fibrinolytic systemoverdose of heparin or dicumarol,liberation of tissue plasminogen activator into the circulation,antibodies against F,thromboplastin,thrombin,heparinoid anticoagulant,DIC.21Classification:Complex disorders of hemostatic mechanismvHereditary or congenitalvWDvAcquiredDICLiver disease22Etiological diagnosis:HistoryvWhoAge SexFamily history vWhenPredisposing cause Drug ingestion?Nutrition statusany concomitant diseases Frequency of the bleeding event 23Etiological diagnosis:Symptoms and signsvWhere:The site of bleeding(skin,mucous membrane,GI tract,solid organ,joint or muscle?)vWhat:The physical characteristics of the bleeding(petechial瘀点瘀点?Purpura紫癜紫癜?Ecchymoses瘀斑瘀斑?hematoma?)vOther concomitant symptoms and signsvVital signs:HR,RR,BP,etc.2425hemophilia26Differential diagnosisTable.The clinical distinction between disorders of vessels and platelets and disorders of blood coagulation 27Etiological diagnosis:Laboratory examinationsvScreening testsFor the Vascular disorders and platelet disorders capillary frangibility test bleeding time(BT)platelet countclot retraction test(CRT)28Etiological diagnosis:Laboratory examinationsFor the disorders of blood coagulationactivated partial thromboplastin time(APTT)prothrombin time(one stage method)(PT)thrombin time(TT)clotting time(CT)293031323334Etiological diagnosis:Laboratory examinationsvConfirmatory testsFor vascular disordersvWFEndothelin-1(ET-1)TM35Etiological diagnosis:Laboratory examinationsvConfirmatory testsFor platelet disordersmegakaryocytes in marrowmorphology of plateletsplatelet adhesion testaggregation test induced by ADP,adrenaline collagen,arachidonic acidanalysis of platelet glycoproteins etc.36Etiological diagnosis:Laboratory examinationsvConfirmatory testsFor disorders of blood coagulation measurement of different factor antigens and coagulant activities37Etiological diagnosis:Laboratory examinationsvConfirmatory testsFor disorders of anticoagulationAT-antigen and activity,TATPCFactor:C antibodyLupus-type anticoagulant,cuorin-type anticoagulant38Etiological diagnosis:Laboratory examinationsvConfirmatory testsFor disorders of fibrinolysis3P testFDPD-dimerPlasminogent-PA39Etiological diagnosis:Laboratory examinationsvSpecial testsFor hereditary disorders or some special disordersProtein structural analysisAmino acid sequencingGene analysisimmunopathology40Etiological diagnosis:diagnostic procedurehemorrhagic disorder?classification(vessel?BPC?Coagulation?)Which factor?quantity or quality?Congenital?Hereditary?Acquired?Gene and molecular biology analysis41TreatmentvEtiological treatment and preventionAcquired diseasesDrugs that impair platelet functions should be avoided.Vigorous treatment of the underlying diseases,such as liver disease.42TreatmentvEtiological treatment and preventionHereditary diseasesNo radical treatment.Avoid intramuscular injections,surgical operation and trauma.Plan surgical procedures carefully,if the operation is inevitable.Antenatal diagnosis43TreatmentvTreatment according to pathophysiologyDrugs decreasing the capillary fragility:Rutin,Vitamin C,adrenosem.Drugs used in thrombocytopenia:adrenal corticosteroid for immunothrombocytopenia.Replacement therapy in coagulation factors deficiencies:Vitamin K in acquired deficiency of Vitamin K dependent factors,coagulation factor transfusion.Antifibrinolysis drugs:para-aminomethylbenzoic acid(PAMBA 氨甲苯酸氨甲苯酸),tranexamic acid(t-AMCHA 氨甲环酸氨甲环酸),-aminocaproic acid(EACA 氨基己酸氨基己酸).Anticoagulants:immunosuppressive drugs for antibodies,protamine sulfate for heparinoid anticoagulants.44TreatmentvOther treatmentAnticoagulation and anti-platelet drugsPlasmapheresissurgery45复习思考题复习思考题v临临床床上上如如何何鉴鉴别别血血小小板板及及血血管管性性疾疾病病、凝凝血血性性疾疾病病所所致致出血?出血?v狼疮性抗凝物的检测及临床意义。狼疮性抗凝物的检测及临床意义。vATAT与肝素在抗凝血中的作用机理。与肝素在抗凝血中的作用机理。v出血性疾病的诊断思路。出血性疾病的诊断思路。v应用肝素抗凝血时,需要监测凝血功能,请问是监测应用肝素抗凝血时,需要监测凝血功能,请问是监测APTTAPTT还是还是PTPT,为什么?,为什么?46
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