七年制出血性疾病课件PPT文档.ppt

Classification of hemorrhagic diseases 1.Abnormality of blood vessel 2.Abnormality of platelets 3.Abnormality of coagulation,Mechanism of cougulation,Coagulation factors,FI，fibrinogenFII，prothrombin FIII，tissue factor，tissue thromboplastinFIV，Ca+FV,labile factorFVII,stable factorFVIII,antihemophilic globulin,AHGFIX,plasma thromboplastin component,PTC，Christmas factorFX,Stuart-Prowe factorFXI,plasma thromboplastin anticedent,PTAFXII,Hageman FactorFXIII,fibrin stablizing factorPKHMWK,Coagulation cascadetheory,The mechanism of anticoagulation and fibrinolysis,The system of anticoagulation*Antithrombin(AT)*Protein C system*Tissue factor pathway inhibitor(TFPI)*Heparin,Fibrinolysis system*Plasminogen(PLG)*t-PA*u-PA*Plasmin-related inhibitor,Coagulation=Anticoagulation,Laboratory examination for hemorrhagic diseases 1.Platelet count 2.Bleeding time(BT)3.Clot retraction test 4.Capillary fragility test If above items are abnormal,that means abnormality of blood vessel or platelet.,5.Clotting time(CT)6.Plasma prothrombin time(PT)7.Thrombin clotting time(TT)8.Activation partial thromboplastin time(APTT)Kaolin partial thromboplastin time(KPTT)If above items are abnormal,that means abnormality of coagulation.,PT FVII deficiencyAPTT hemophilia or FXI deficiency PT deficiency of FV,FX,APTT FII,or fibrinogen abnormalities.,Acquired deficiencies of plasma coagulation are more frequent than congenital disorder;the most common disorders include:-Hemorrhagic diathesis of liver disease;-Disseminated intravascular coagulation(DIC),-Vitamin K deficiency of more than one clotting factor.,treatment,Requires replacement of the deficient protein using recombinant or purified plasma derived products or fresh plasma.,Disseminated Intravascular Coagulation,DIC,Definition,The syndrome of DIC is a pathological state in the development of diseases.DIC is always secondary to another disorder.It never occurs as a primary disease.,Etiology,Infectious diseases:43%*bacteria infection*virus*protozoon malaria,Etiology,Malignant tumor:34%,Pathologic obsterics:12%Operation and trauma:5%Systemic disease,Pathogenesis,1.Damage of tissue release of tissue factor into blood activate extrinsic coagulation pathway2.Damage of vascular epitheliaintrinsic coagulation system3.Platelet activate4.Activate fibrinolysindisturbance of coagulation and fibrinolysis,CoagulationAnticoagulation CoagulationAnticoagulationThe disturbance of balance between coagulation and anticoagulation,Pathology and pathophysiology,Microthrombosis-the fundamental and specific change of pathologyAbnormality of coagulation*hypercoagulable stage*consumptive hypocoagulable stage*secondary hyperfibrinolytic stageDisturbance of microcirculation,Clinical manifestations,Bleeding tendencyShock or disturbance of microcirculationEmbolism of microvasculatureMicroangiopathic hemolysisManifestations of primary disease.,Clinical manifestations,Bleeding tendencyShock or disturbance of microcirculationEmbolism of microvasculatureMicroangiopathic hemolysisManifestations of primary disease.,Clinical manifestations,Bleeding tendencyShock or disturbance of microcirculationEmbolism of microvasculatureMicroangiopathic hemolysisManifestations of primary disease.,DIC,Clinical manifestations,Bleeding tendencyShock or disturbance of microcirculationEmbolism of microvasculatureMicroangiopathic hemolysisManifestations of primary disease.,Lab examination,1.platelet count2.quantitative of plasma fibrinogen4g/L3.3p test(+)or plasma FDP 20mg/L(60mg/L in liver disease)or D-dimer(+)or increased4.PT:prolonged or shorten more than 3s(in liver disease 5s)or dynamic change5.plasminogen decreased 6.AT III decreased.7.plasma FVIII:C 50%(in liver disease it must be+),Diagnosis1.Primary disease of DIC2.At least 2 items of clinical manifestation.Anticoagulant treatment is effective3.At least 3 items of lab examination(+),Treatment,Eliminate the inducing factors and causes of DIC and treat primary diseaseAnticoagulation therapy:Heparin-APTT prolonged 60%-100%is very goodReplenish coagulation factors and plateletsAntifibrinolysis therapy:EACA PAMBA,Idiopathic thrombocytopenic purpura(ITP),also known as Primary immune thrombocytopenia(ITP),Etiology and Pathogenesis(1)Infection(2)Immunity factors:PA IgG and PB IgG(3)Spleen factor(4)Other factor:estrogen,Clinical manifestation,(1)Onset Acute type Chronic type 1)Children women 2)Abrupt insidious 3)History of upper(-)respiratory tract infection 4)Petechiae menorrhea Organ bleeding 5)PLT 20109/L 50109/L 左右,(2)Hemorrhagic symptompetechiae,purpura,hematuriagastrointestinal tract hemorrhagegum bleedingmenorrheaintracranial hemorrhage,(3)Sign 1)Purpura2)The spleen usually can not be palpable or enlargement,Laboratory examination(1)Platelet count Acute ITP 20109/L Chronic ITP 30-80109/L(2)BT is prolongation Clot retraction is impaired.Capillary fragility is positive.CT is normal.(3)Bone marrow:The number of megakaryocytes are increased or normal with maturation disturbance.,Diagnosis1.Extensive bleeding2.Repeated examinations reveal that platelet count is decreased in peripheral blood.3.Bone marrow4.No hepatosplenomegaly5.Cortisone treatment or splenectomy is effective6.Rule out other diseases:Leukemia,AA,ITP,SLE,tumor,Treatment1.Glucocorticoid:first choice 1)Prednisone:1mg kg.d gradually decreased 2)Dexamethasone:10 20mg intravenous drip 3)Methylprednisolone:120-500mg/d,iv drop,(2)Splenectomy can be done:Indication:1)Glucocorticoid treatment for 36 months is not effective.2)Small dosage is easily to relapse.prednisone must 30mg/day.3)The use of glucocorticoid is contraindicated.4)Isotope labeled platelet increases in spleen.,(3)Immunosuppression:46WS 1)VCR:1mg once each week.iv or iv drop 3 6WS 2)CTX 3)6MP 4)CsA 5)Rituximab,(4)Severe cases:PLT20 109/L Severe and extensive bleedingintracranial hemorrhage4)operation at present,Treatment of severe cases,1)supportive treatments PLT or fresh blood transfusion in severe cases,PLT 20 109/L 2)Gamma globulins:0.20.4/kg.d,5 days,iv drop3)Plasma change:PA IgG decrease4)large dosage methylprednisolone:1.o g/d.iv drop.3 5days.,Experimental and novel agents,H.pylori eradicationAnti-DDapsone TPOThrombopoietin Receptor Agonists:,Romiplostim,Eltrombopag,Key point,Common Causes of DICInfection disease(Gram-negative sepsis)Malignant tumor(leukemia,lymphoma,cancers of liver,lung,pancreas,prostate and stomach)Obstetric complications(abruption placentae,pre-eclampsia,amniotic fluid embolism)Operation and traumaSystemic disease,Key point,Laboratory tests for DIC diagnosis Platelet counts100109/L,or a rapid declineFibrinogen1.5g/L,4g/L,or a rapid declineFibrin degradation product(FDP)20mg/L,and/or D-dimer increaseProlongation or shorten of PT(more than 3s)and/or APTT(more than 10s),Key point,Which situation cannot you use antifibrinolytic drugs,such as EACA?hematuria,Key point,Treatment of ITPPlatelet transfusionSteriodsIVIgSplenectomyImmunosuppresantsThrombopoietin Receptor Agonists,谢 谢！,