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1、Classification of hemorrhagic diseases 1. Abnormality of blood vessel 2. Abnormality of platelets 3 . Abnormality of coagulation,Mechanism of cougulation,Coagulation factors,FI，fibrinogen FII，prothrombin FIII，tissue factor，tissue thromboplastin FIV，Ca+ FV, labile factor FVII, stable factor FVIII, an
2、tihemophilic globulin, AHG FIX, plasma thromboplastin component,PTC， Christmas factor FX, Stuart-Prowe factor FXI, plasma thromboplastin anticedent,PTA FXII, Hageman Factor FXIII, fibrin stablizing factor PK HMWK,Coagulation cascade theory,The mechanism of anticoagulation and fibrinolysis,The system
3、 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)
4、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(
5、KPTT) If above items are abnormal, that means abnormality of coagulation.,PT FVII deficiency APTT 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 disor
6、ders 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 I
7、ntravascular 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 tu
8、mor : 34%,Pathologic obsterics: 12% Operation and trauma: 5% Systemic disease,Pathogenesis,1.Damage of tissue release of tissue factor into blood activate extrinsic coagulation pathway 2.Damage of vascular epitheliaintrinsic coagulation system 3.Platelet activate 4.Activate fibrinolysindisturbance o
9、f coagulation and fibrinolysis,CoagulationAnticoagulation CoagulationAnticoagulation The disturbance of balance between coagulation and anticoagulation,Pathology and pathophysiology,Microthrombosis-the fundamental and specific change of pathology Abnormality of coagulation *hypercoagulable stage *co
10、nsumptive hypocoagulable stage *secondary hyperfibrinolytic stage Disturbance of microcirculation,Clinical manifestations,Bleeding tendency Shock or disturbance of microcirculation Embolism of microvasculature Microangiopathic hemolysis Manifestations of primary disease.,Clinical manifestations,Blee
11、ding tendency Shock or disturbance of microcirculation Embolism of microvasculature Microangiopathic hemolysis Manifestations of primary disease.,Clinical manifestations,Bleeding tendency Shock or disturbance of microcirculation Embolism of microvasculature Microangiopathic hemolysis Manifestations
12、of primary disease.,DIC,Clinical manifestations,Bleeding tendency Shock or disturbance of microcirculation Embolism of microvasculature Microangiopathic hemolysis Manifestations of primary disease.,Lab examination,1.platelet count 2.quantitative of plasma fibrinogen4g/L 3.3p test (+) or plasma FDP 2
13、0mg/L(60mg/L in liver disease) or D-dimer (+) or increased 4.PT: prolonged or shorten more than 3s ( in liver disease 5s) or dynamic change 5.plasminogen decreased 6.AT III decreased. 7.plasma FVIII:C 50%(in liver disease it must be +),Diagnosis 1. Primary disease of DIC 2. At least 2 items of clini
14、cal manifestation. Anticoagulant treatment is effective 3. At least 3 items of lab examination(+),Treatment,Eliminate the inducing factors and causes of DIC and treat primary disease Anticoagulation therapy : Heparin-APTT prolonged 60%-100% is very good Replenish coagulation factors and platelets An
15、tifibrinolysis 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
16、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 symptom petechiae , purpura, hematuria gastrointestinal tract hemorrhage gum bleeding menorrhea intracranial hemorrha
17、ge,(3)Sign 1) Purpura 2) 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 megakary
18、ocytes are increased or normal with maturation disturbance.,Diagnosis 1.Extensive bleeding 2.Repeated examinations reveal that platelet count is decreased in peripheral blood. 3.Bone marrow 4.No hepatosplenomegaly 5.Cortisone treatment or splenectomy is effective 6.Rule out other diseases: Leukemia,
19、 AA, ITP, SLE, tumor,Treatment 1.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
20、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 an
21、d extensive bleeding intracranial hemorrhage 4) 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 drop 3) Plasma change: PA IgG decrease 4) large dosage methylprednisolone:
22、1.o g/d. iv drop. 3 5days.,Experimental and novel agents,H. pylori eradication Anti-D Dapsone TPO Thrombopoietin Receptor Agonists:, Romiplostim, Eltrombopag,Key point,Common Causes of DIC Infection disease (Gram-negative sepsis) Malignant tumor (leukemia, lymphoma, cancers of liver, lung, pancreas,
23、 prostate and stomach) Obstetric complications (abruption placentae, pre-eclampsia, amniotic fluid embolism) Operation and trauma Systemic disease,Key point,Laboratory tests for DIC diagnosis Platelet counts100109/L, or a rapid decline Fibrinogen1.5g/L, 4g/L, or a rapid decline Fibrin degradation pr
24、oduct (FDP)20mg/L, and/or D-dimer increase Prolongation 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 ITP Platelet transfusion Steriods IVIg Splenectomy Immunosuppresants Thrombopoietin Receptor Agonists,谢 谢 ！,