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Hemorrhagic Disorders

Hemorrhagic Disorders. Huang Honghui Dept. of Hematology, Renji Hospital. Definition. When the normal hemostatic mechanism fails, major hemorrhage may follow minor trauma or may arise apparently spontaneously. Mechanism of normal hemostasis. Vascular mechanism Platelet mechanism

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Hemorrhagic Disorders

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  1. Hemorrhagic Disorders Huang Honghui Dept. of Hematology, Renji Hospital

  2. Definition • When the normal hemostatic mechanism fails, major hemorrhage may follow minor trauma or may arise apparently spontaneously.

  3. Mechanism of normal hemostasis • Vascular mechanism • Platelet mechanism • Blood coagulation mechanism

  4. Normal hemostasis

  5. red thrombus

  6. Anticoagulation and fibrinolystic mechanism • Anticoagulation system • AT-Ⅲ • Protein C system: PC, PS, TM • TFPI • Heparin

  7. Anticoagulation and fibrinolystic mechanism • Fibrinolysis system • Plasminogen • Tissue-type plasminogen activator(t-PA) • Urokinase- type plasminogen activator(u-PA) • Inhibitors:α2-plasmin inhibitor(α2-PI), plasminogen activator inhibitor-1(PAI-1)

  8. Anticoagulation and fibrinolystic mechanism Plasminogen ActivatorInhibitor Kallekrein (intrinsic) t-PA,u-PA PAI (extrinsic) α2-PI plasmin fibrinogen(fibrin) FDP

  9. Classification • vascular disorders • platelet disorders • disorders of blood coagulation • disorders of anticoagulation system and fibrinolytic system • Complex disorders of hemostatic mechanism

  10. Classification: Vascular disorders • Hereditary or congenital: Hereditary hemorrhagic telangiectasia（遗传性出血性毛细血管扩张症）, hereditary disorders of connective tissue such as Ehlers-Danlos syndrome. • Secondary: infections, chemical factors or drugs, disorders of metabolism (Vitamin C or P deficiency), pathological changes of vascular wall (atherosclerosis), connective tissue diseases. • Allergy: allergic purpura. • Other purpuras: purpura simplex, senile purpura, mechanic purpura, paraproteinemia.

  11. Classification: platelet disorders Thrombocytosis Thrombocytopenia Thrombocytopathy

  12. Classification: platelet disorders

  13. Classification: platelet disorders • Thrombocytosis • Primary: essential thrombocythemia • Secondary: infections, injury, post-splenectomy, chronic myelocytic leukemia, other myeloproliferative disorders (such as polycythemia vera 真性红细胞增多症）)

  14. Classification: platelet disorders • Functional abnormalities of platelets • Hereditary: giant platelet syndrome (Bernard-Soulier syndrome),thrombasthenia(血小板无力症）, • Acquired: due to drugs, uremia, liver diseases, dysproteinemias.

  15. Classification: disorders of blood coagulation • Hereditary or congenital: hemophilia A (F Ⅷ deficiency), hemophilia B (F Ⅸ deficiency), factor Ⅺ deficiency (formerly hemophilia C), hypothrombinogenemia, hypofibrinogenemia, von Willebrand’s disease, other coagulation factors deficiency, including deficiency of activated-protein C inhibitor structural abnormalities. • Acquired: Vitamin K deficiency, severe liver diseases, disseminated intravascular coagulation (DIC) etc.

  16. Classification: disorders of anticoagulation system and fibrinolytic system • disorders of anticoagulation system and fibrinolytic system • overdose of heparin or dicumarol, liberation of tissue plasminogen activator into the circulation, antibodies against FⅧ, Ⅸ, thromboplastin, thrombin, heparinoid anticoagulant, DIC.

  17. Classification: Complex disorders of hemostatic mechanism • Hereditary or congenital • vWD • Acquired • DIC • Liver disease

  18. Etiological diagnosis: History • Who • Age • Sex • Family history • When • Predisposing cause • Drug ingestion? • Nutrition status • any concomitant diseases • Frequency of the bleeding event

  19. Etiological diagnosis:Symptoms and signs • Where: • The site of bleeding (skin, mucous membrane, GI tract, solid organ, joint or muscle? ) • What: • The physical characteristics of the bleeding (petechial瘀点? Purpura紫癜? Ecchymoses瘀斑? hematoma? ) • Other concomitant symptoms and signs • Vital signs: HR, RR, BP,etc.

  20. Differential diagnosisTable. The clinical distinction between disorders of vessels and platelets and disorders of blood coagulation

  21. Etiological diagnosis:Laboratory examinations • Screening tests • For the Vascular disorders and platelet disorders • capillary frangibility test • bleeding time(BT) • platelet count • clot retraction test(CRT)

  22. Etiological diagnosis:Laboratory examinations • For the disorders of blood coagulation • activated partial thromboplastin time (APTT) • prothrombin time (one stage method) (PT) • thrombin time (TT) • clotting time (CT)

  23. Etiological diagnosis:Laboratory examinations • Confirmatory tests • For vascular disorders • vWF • Endothelin-1(ET-1) • TM

  24. Etiological diagnosis:Laboratory examinations • Confirmatory tests • For platelet disorders • megakaryocytes in marrow • morphology of platelets • platelet adhesion test • aggregation test induced by ADP, adrenaline collagen, arachidonic acid • analysis of platelet glycoproteins etc.

  25. Etiological diagnosis:Laboratory examinations • Confirmatory tests • For disorders of blood coagulation • measurement of different factor antigens and coagulant activities

  26. Etiological diagnosis:Laboratory examinations • Confirmatory tests • For disorders of anticoagulation • AT-Ⅲ antigen and activity, TAT • PC • Factor Ⅷ:C antibody • Lupus-type anticoagulant, cuorin-type anticoagulant

  27. Etiological diagnosis:Laboratory examinations • Confirmatory tests • For disorders of fibrinolysis • 3P test • FDP • D-dimer • Plasminogen • t-PA

  28. Etiological diagnosis:Laboratory examinations • Special tests • For hereditary disorders or some special disorders • Protein structural analysis • Amino acid sequencing • Gene analysis • immunopathology

  29. Etiological diagnosis:diagnostic procedure hemorrhagic disorder? classification(vessel? BPC? Coagulation?) Which factor? quantity or quality? Congenital? Hereditary? Acquired? Gene and molecular biology analysis

  30. Treatment • Etiological treatment and prevention • Acquired diseases • Drugs that impair platelet functions should be avoided. • Vigorous treatment of the underlying diseases, such as liver disease.

  31. Treatment • Etiological treatment and prevention • Hereditary diseases • No radical treatment. • Avoid intramuscular injections, surgical operation and trauma. • Plan surgical procedures carefully, if the operation is inevitable. • Antenatal diagnosis

  32. Treatment • Treatment according to pathophysiology • Drugs 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), tranexamic acid (t-AMCHA 氨甲环酸),ε-aminocaproic acid (EACA 氨基己酸). • Anticoagulants: immunosuppressive drugs for antibodies, protamine sulfate for heparinoid anticoagulants.

  33. Treatment • Other treatment • Anticoagulation and anti-platelet drugs • Plasmapheresis • surgery

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