Hemorrhagic syndrome

1. VinnytsyaNational Pirogov Memorial Medical University Pathophysiology Department Hemorrhagic syndrome PhD., Viktoriya Piliponova

2. System of hemostasisis the complex of mechanisms which is directed on liquidation of blood loss after damage of blood vessels by formation of blood clots, maintaining of liquid condition of blood in norm. • Hemostasis is a normal physiological response that prevents significant blood loss after vascular injury

3. Hemostasis divided in two parts: • Vascular or primary • Plasmatic or secondary

4. Classification of hemorrhagic diathesis according etiology: • Acquired - after using of cytostastic therapy and aspirin, action of ionizing radiation, autoimmune mechanisms. • Inherited - hemophilia A, B, C, Von Willebrand’s disease.

5. Pathogenic classification : • Violation of thrombocytes • Pathology of vessels • Violation of coagulation system • Violation of anti-coagulation system • Combined violation (DIC syndrome)

6. Petechiae: • Small tiny red dots • Small, 1-2 mm by size hemorrhages in skin and mucus membrane • Petechiae occurs with increased pressure, thrombocytopenia due to violation of angiotrophic function of thrombocytes, insufficiency of vitamin C

7. Purpura: • Like mosquito bites • Middle, 3mm by size hemorrhages mostly connected with vasculitis and trauma

8. Ecchymoses: • Large, 1-2 cm by size • Connected with trauma, vasculitis or other conditions

9. Hematomas: • Massive, deep and painful accumulation of blood in joints, muscles, mucous membrane • Connected with violation of coagulation stage of hemostasis (hemophilia)

10. Laboratory tests for patients with hemorrhagic syndrome: • Level of thrombocytes – in norm - 200-400 x 109/l • Time of bleeding (Duke test) - 2- 4 min. • Time of blood coagulation by Li-Wait ( norm 4-9 min) • Capillary resistance test ofHess(«-» - negative) • Prothrombin time (PT) - 9,8-12,1sec. • Thrombin time (TT) -14-21 sec. • Activated partial thromboplastin time (АPТT) - 22,7-31,8 sec.

11. Function of thrombocytes • Angiothrophic function • Formation of primary blood clots • Maintaining of vasospasm in damaged vessels • Taking place in blood coagulation and fibrinolysis

12. Thrombocytopenia: is the decreasing of thrombocytes level in peripheral blood, less than 150 x 109/L Thrombocytopenia may be: • Absolute and relative • Acquired or inherited

14. Classification of thrombocytopenias accoding pathogenesis: • Decreased thrombocytes synthesis in the bone marrow • Increased thrombocytes destruction • Increased thrombocyte using for blood clot formation (DIC syndrome)

15. Autoimmune idiopathic thrombocytopenic purpura, (AITP), Werlgoff’s disease • immune destruction of thrombocyte (formation of antithrombocytic antibodies), functional changes in megakaryocytic sprout of hemopoiesis, breaking action of spleen on the bone marrow.

17. Clinical features AITP • Spontaneous repeated petechia, • nasal, GIT - bleeding, • hemorrhages of different colors (from red to yellowish, depends on time and origin – leopard skin symptom) • microhematuria.

19. AITP (Werlgoff’s disease) Laboratory test: • Thrombocytes – decrease. • The BT (bleeding time) is prolonged. • PT and APTT are normal. Treatment: • corticotherapy, • splenectomy, • immunosuppressive drugs.

20. THROMBOCYTOPATHY′S - violation of thrombocytes functions and normal level of them in peripheral blood. Divided into: • Inherited type; • Acquired type.

21. Classification of thrombocythopathy’s according pathogensis: • enzymopathy (G-6-PDH deficiency); • membranopathy (deficiency of the membrane receptors of the V, VIII, factors); • violation of granules (granulopathia)

22. Von Willebrand disease (angiohemophilia, violation of adhesion) • It is a genetic disorder that is inherited as an autosomal recessive trait, connected with defects of synthesis endothelial factor of adhesion, characterized by mucosal, nasal bleeding

23. Von Willebrand disease Laboratory test: • Thrombocytes level in peripheral blood in norm. • On blood smear - large platelets. • BTis prolonged. • Deficiency of factor VIII, and impaired platelet adhesion. • Treatment: Platelet transfusion is necessary to control serious bleeding

24. Vasopathy: is the acquired or genetic defect, connected with damage or violation of vessels wall. Classification of vasopathy: • By etiology: • Inherited (hemangiomas) • Acquired (vasculitis, hypovitaminosis) • By mechanism: • inflammatory • dysplastic

26. SCHONLEIN - HENOCH SYNDROME - immune vasculitis induced 2-3 weeks after an infection with streptococcus β-hemolytic, characterized by widely spread petechiae and blood clots formation in small vessels.

27. Clinical forms: • Simple (skin) form – numerous symmetric small petechia on extremities, abdomen, itching.

28. Abdominal form – grave form, characterized by intoxication, hemorrhages in intestinal wall or vessel of internal organs.

29. Joints form – characterized by middle skin petechia and pain in big joints

30. Dysplastic vasopathy - connected with inherited or acquired violation of collagen synthesis in vessel walls. • Reasons: • Vitamine C deficiency • Hereditary haemorrhagic telangiectasia

31. Coagulopathy:hemorrhagic syndrome, connect with violation of coagulation and anticoagulation mechanism. • Classification: by etiology: • inherited (hemophilia A,B,C) • acquired (insufficiency of Vitamine K)

32. Classification сoagulopathy by pathogenesis: I.Violations activation external mechanism of coagulation II. Violations of the activation internal mechanism of the coagulation: • Disorders of lst phase of blood coagulation: • Deficiency of f.VIII, f. IX, f. XI • Thrombocytopenia, thrombocytopathy • Angiohemophilia (Willebrand’s disease). • Disorders of 2nd phase of blood coagulation: • Deficiency f. II, f.V, f.VII, f.X. • Disorders of 3rd phase of blood coagulation: • Deficiency of f. I, f. XII, reduction of fibrinogen synthesis. III. Hémorragie diathesis, resulted by fast fibrinolysis. IV. Combinet hémorragic syndrom (DIC syndrome)

33. Hemophilia A is caused by a mutation in the gene for factor VIII, so there is deficiency of this clotting factor.

34. Hemophilia A: Clinical manifestations: • spontaneous bleeding • Bruises and hematoma • Bleeding into joints • GIT and urinary hemorrhage → blood in stool and urine • Prolonged bleeding after tooth extraction, from cuts ! Never purpura and petechiae (since primary hemostasis is not affected)

35. DISSEMINATED INTRAVASCULAR COAGULATION (DIC) - is a disorder of hemostasis, which manifests itself through massive formation of thrombi and microemboli in the vessels, mainly of the microcirculatory bed.

36. Etiology: • massive trauma of tissues, Crush syndrome, operative trauma of the parenchymatous organs; • massive burn; • generalized infections, sepsis; • all kinds of shock; • obstetrical pathology, amniotic fluid embolism; • malignancies • toxic (after snake bite); • allergic • infectious vasculitis • hemolytic transfusion reactions • uremia (renal insufficiency); • peritonitis;

37. In the pathogenesis of DIC distinguish phases: • I stage of hypercoagulation • II stage of hypocoagulation. • Ill stage - outcomes

40. Thromboelastogram (TEG) is a graphical study of different hemostasis indices.

41. Дякую за увагу!!