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1、Hemorrhagic diseases MD & PHD Professor Aijun Liao Department of Hematology Shengjing Hospital of China Medical UniversityCell phone: 18940259833;Classification of hemorrhagic diseases 1. Abnormality of blood vessel 2. Abnormality of platelets 3 . Abnormality of coagulation;Mechanism of cougulation;
2、Coagulation factors FIFI，fibrinogenfibrinogenFIIFII，prothrombin prothrombin FIIIFIII，tissue factortissue factor，tissue thromboplastintissue thromboplastinFIVFIV，Ca+Ca+FV, labile factorFV, labile factorFVII, stable factorFVII, stable factorFVIII, antihemophilic globulin, AHGFVIII, antihemophilic glob
3、ulin, AHGFIX, plasma thromboplastin component,PTCFIX, plasma thromboplastin component,PTC， Christmas factor Christmas factorFX, Stuart-Prowe factorFX, Stuart-Prowe factorFXI, plasma thromboplastin anticedent,PTAFXI, plasma thromboplastin anticedent,PTAFXII, Hageman FactorFXII, Hageman FactorFXIII, f
4、ibrin stablizing factorFXIII, fibrin stablizing factorPKPKHMWK HMWK ;Coagulation cascadetheory;The mechanism of anticoagulation and fibrinolysisThe system of anticoagulation *Antithrombin (AT) *Protein C system *Tissue factor pathway inhibitor (TFPI) *Heparin;Fibrinolysis system *Plasminogen (PLG) *
5、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
6、 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
7、, 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 clo
8、tting factor.;treatmentRequires replacement of the deficient protein using recombinant or purified plasma derived products or fresh plasma. ;Disseminated Intravascular CoagulationDIC;DefinitionThe syndrome of DIC is a pathological state in the development of diseases. DIC is always secondary to anot
9、her disorder. It never occurs as a primary disease.;EtiologyInfectious diseases: 43% *bacteria infection *virus *protozoon malaria;EtiologyMalignant tumor : 34%;Pathologic obsterics: 12%Operation and trauma: 5%Systemic disease;Pathogenesis1.Damage of tissue release of tissue factor into blood activa
10、te 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;Path
11、ology and pathophysiologyMicrothrombosis-the fundamental and specific change of pathologyAbnormality of coagulation *hypercoagulable stage *consumptive hypocoagulable stage *secondary hyperfibrinolytic stageDisturbance of microcirculation;Clinical manifestationsBleeding tendencyShock or disturbance
12、of microcirculationEmbolism of microvasculatureMicroangiopathic hemolysisManifestations of primary disease.;Clinical manifestationsBleeding tendencyShock or disturbance of microcirculationEmbolism of microvasculatureMicroangiopathic hemolysisManifestations of primary disease.;Clinical manifestations
13、Bleeding tendencyShock or disturbance of microcirculationEmbolism of microvasculatureMicroangiopathic hemolysisManifestations of primary disease.;DIC;Clinical manifestationsBleeding tendencyShock or disturbance of microcirculationEmbolism of microvasculatureMicroangiopathic hemolysisManifestations o
14、f primary disease.;Lab examination1.platelet count2.quantitative of plasma fibrinogen 1.5g/L or dynamic decrease, in liver disease 1.0g/L( in malignant tumor 4g/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
15、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(+) ;TreatmentElimin
16、ate 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 thrombocy
17、topenia (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
18、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
19、 Acute ITP 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
20、hemorrhage4) operation at present;Treatment of severe cases1) 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.;Experimenta
21、l and novel agentsH. pylori eradicationAnti-DDapsone TPOThrombopoietin Receptor Agonists:, Romiplostim, Eltrombopag;Key pointCommon Causes of DICInfection disease (Gram-negative sepsis)Malignant tumor (leukemia, lymphoma, cancers of liver, lung, pancreas, prostate and stomach)Obstetric complications
22、 (abruption placentae, pre-eclampsia, amniotic fluid embolism)Operation and traumaSystemic disease;Key pointLaboratory 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 pointWhich situation cannot you use antifibrinolytic drugs, such as EACA?hematuria;Key pointTreatment of ITPPlatelet transfusionSteriodsIVIgSplenectomyImmunosuppresantsThrombopoietin Receptor Agonists ;谢谢 谢谢 ！;
