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Pathology of h а emostasis

Pathology of h а emostasis. Ph.D. , MD , Assistant Professor Dzyha Svitlana Viktorivna. Meaning. 1. Control of blood fluid condition ( is due to adequate correlation between activity of coagulative blood system and anticoagulative one )

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Pathology of h а emostasis

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  1. Pathology of hаemostasis Ph.D., MD,Assistant Professor Dzyha Svitlana Viktorivna

  2. Meaning • 1. Control of blood fluid condition (is due to adequate correlation between activity of coagulative blood system and anticoagulative one) • 2. Bleeding stop(is very important for control of the blood circulation index)

  3. HAEMOSTASIS SYSTEMS • VESSEL-TROMBOCYTE’S (PRIMARY HAEMOSTASIS) - Bleeding stopin microvessels • COAGULATIVE (SECONDARY) FIBRIN CLOTS FORMATION

  4. HAEMOSTASIS SYSTEMS COMPONENTS • * VESSEL’S WALL • * ТROMOCYTES • * SYSTEMS OF THE BLOOD PLASMA: • - COAGULATIVE • - ANTICOAGULATIVE • - FIBRINOLYSIS • - KALLIKREIN-KININ

  5. НАEMOSTASISVIOLATIONCLASSIFICATION • BY ETHIOLOGY - HEREDITARY - ACQUIRED • BY PATHOGENESIS - VESSEL-TROMBOCYTE’SНАEMOSTASIS VIOLATION - COAGULATIVEНАEMOSTASISVIOLATION • BY DIRECTION OF THE CHANGES • - HYPOCOAGULATION - HYPERCOAGULATION

  6. HYPOCOAGULATION • REDUCED PROPERTYOF THE BLOODTO FORM OF THE BLOOD CLOTS which results in spontaneous bleeding or hemorrhage • (very often after trivial trauma)

  7. ETHIOLOGY • 1. THROMBOCYTOPENIA • 2. THROMBOCYTOPATHY • 3. VASOPATHY • 4. CОАGULOPATHY

  8. THROMBOCYTOPENIA Pathological condition which is characterized by the decreased amount of thrombocytes in the blood (LESS THEN150·109/l)

  9. HEREDITARYTHROMBOCYTOPENIA • As a rule, is accompanied with hereditary thrombocytes defect

  10. ACQUIREDTHROMBOCYTOPENIA • REASONS • INJURY OF THE THROMBOCYTES - by immune complexes - mechanical trauma(at spleenmegaly, haemangioma) • THROMBOCYTESMATURATION DEPRESSION (aplastic anemia, red bone marrow injury by chemical poisons and radiation, tumors in bone marrow – metastasis or primary) • ACCELERATED USING OF THE THROMBOCYTES (thrombosis, DIC-syndrome)

  11. IMMUNETHROMBOCYTOPENIA • HETEROIMMUNE Arises most often in children Reason – change of thrombocyte’s antigen structure (attachmentof the viruses at measles, smallpox; attachment of the medicine’s origin haptens; vaccines) Clinical course is benign(elimination of the reason conduces to absolute recovery)

  12. IMMUNETHROMBOCYTOPENIA • AUTOIMMUNE Arises most often in adult Reason – no immune system tolerance to own thrombocyte’s antigens Provoked agents: medicines, viruses, bacteria

  13. AUTOIMMUNE THROMBOCYTOPENIA WERLGOFF’S disease 1. Ig G amount on the thrombocite’s surface increases in 10 time 2. main place of Ig G synthesis is spleen 3. treatment principles: - spleenectomy - corticosteroids - depression of immune responce by medicines * Absolut recovery is not

  14. THROMBOCYTOPATY • НАEMOSTASISVIOLATION AS THE RESULTOF QUOLITATIVE THROMBOCYTE’S DEFECTS ORTHROMBOCYTE’S DYSFUNCTION,WHICH IS CHRACTERISE BY VESSEL-TROMBOCYTE’SНАEMOSTASIS VIOLATION, APPERENCE OF HAEMORHAGIES IN TISSUES AND ORGANS

  15. HEREDITARYTHROMBOCYTOPATY • WITHOUT VIOLATION OF GRANULES EXCRETION GLANCMAN’S disease *Type of inheritance - autosomal-recessive *Reason - noglycoproteins2в and 3аin thrombocite’s membrane *Pathogenesis– thrombocytes cannot interact with fibrinogen,so they don’t aggregate *Signs: skin hemorrhages, nasal bleeding, uterine bleeding (some time deadly!!)

  16. HEREDITARYTHROMBOCYTOPATY • BECAUSE EXCRETION OF GRANULES IS VIOLATIED Type of inheritance - autosomal-recessive Reason – violation of cycleoxigenase activity, low activity of contractive proteins Pathogenesis – no thrombocite’s agregation at collagen contact, no granules excretion Signs: skin hemorrhages, nasal bleeding, uterine bleeding (some time deadly!!)

  17. HEREDITARYTHROMBOCYTOPATY • DEFICIT OFGRANULES AND THEIR CONTENT • HERDJMANSKY-PUDLAK’S DISEASE (AR) • Reason– deficit of the dense granules (АDP, аadrenalin, serotonin, Са2+) • Pathogenesis – no thrombocytes aggregation after contact with collagen, no excretion of the granules substances • Signs: skin hemorrhages, nasal bleeding, uterine bleeding

  18. HEREDITARYTHROMBOCYTOPATY • VIOLATION OF THROMBOCYTE’S ADGESION AND AGGREGATION • Willebrand-Jurgens’s syndrome (Аr) • Reason –Willebrand’s factor deficit • Pathogenesis – violation of thrombocytes adgesion because activity of c.f.8 is reduced • Bernar-Sulie’s disease (АR) • Reason – noglycoproteins1in thrombocite’s membrane • Pathogenesis – is violated interaction between thrombocytes and f.Willebrand, f. 5, f. 11 • Signs – capillary bleeding (particularly during sex aging and delivery)

  19. HEREDITARYTHROMBOCYTOPATY THROMBOCYTE’S FACTOR 3 DEFICIT • BOUE-OWEN’S disease • Reason – f.3deficit in thrombocytes • Pathogenesis – nointeraction between thrombocytes and coagulative plasma factors • Signs: skin hemorrhages, nasal bleeding, uterine bleeding

  20. HEREDITARYTHROMBOCYTOPATY • COMBINATION OF THROMBOCYTOPATIA andOTHERHEREDITARYANOMALIES • VISCOTT-ОLDREEG’Ssyndr. - Reason – reduced amount in thrombocytes dense granules (АDФ, аadrenalin, serotonin, Са2+)and alpha- granules(beta-thrombglobulin, fibrinogene, fibronectin, growth factor) - Pathogenesis –weak adgesion and aggregation of the thrombocytes, no granules excretion - Signs: hemorrhage syndrome appears in childhood (deadly bleeding is possible)

  21. ACQUIREDTHROMBOCYTOPATY(ETIOLOGY) • 1. Leucaemia – granules deficit inside thrombocytes because their maturation is accelerated (reduced adhesion and aggregation) • 2. Ig М accumulation(causes injuryof thrombocyte’s receptors and violation of thrombocytes and coagulative factors interaction(at imune diseases) • 3. V.В12deficit(causes violation of granules excretion) • 4.Medicines

  22. Medicine’s THROMBOCYTOPATY * ThromboxanА2synthesis inhibitors -antiinflamational steroid medicines - antiinflamational nonsteroid medicines(aspirin inhibitsthrombocytes aggregation, effect last 4-6 days) * Stimulation of cAMP synthesis -papaverin -euphillin -anabolic steroids * Са-ions blockers -verapamil -corinphar

  23. VASOPATHY • Hemorrhage diathesiscaused byfunctional and morphologicaldefect of vessel’s wall HEREDITARY ACQUIRED

  24. HEREDITARYVASOPATHY • RANDIU-ОSLER’S dis., FABRE’Sdis. • Reason –violation of connective tisue development in vessel’s subendothelium - local thickening of the vessels - microvessel’sdilation - little of collagen fibres in subendothelium - vesselsare very easy traumatized - weak adgesion end aggregation of the thrombocytets as a result of collagen fibres deficit Signs: –nasal bleeding, pulmonary-bronchial bleeding, gastro-intestinal bleeding(some timedeadly)

  25. ACQUIRED VASOPATHY 1. Idiopathial(Caposhi’s sarcoma) - ethiology – unknown 2. Stagnantal(Klotc’s dermatitis, Fawr-Rakusho’sdermatitis) - ethiology – chronic heart failure, localvenousinsufficiency 3. Distrophial - uprarenal glands hyperfunction, glucocorticoid therapy(steroid purpura - occures because collagen fibres synthesis depression) - vitamin C deficit - vessel’s damage by immune complexex (Shenliayn-Ganouh’s diseas) Signs: – scin haemorrhagies

  26. CОАGULOPATHY • Pathologacal state which results fromdeficite of coagulative system activity HEREDITARY ACQUIRED

  27. HEREDITARYCОАGULOPATHY • Group of the diseases caused by hereditary deficit or hereditary molecular anomaly of the substancies which control of blood coagulative properties

  28. HEREDITARYCОАGULOPATHY • CLASSIFICATION 1. Coagulopathia as a result of isolated deficit of internal mechanism of prothrombinase activation(haemophylia А, В, С, Willebrand’s dis.,Hageman’s deficit) 2. Coagulopathia as a result of isolated deficit of external mechanism of prothrombinase activation(hypoproconvertinemia – deficit of 7 c. f.) 3. Combined deficit of internal mechanism and external one of prothrombinase activation(parahaemophylia – deficit of 5 c. f., Stuart-Prauer’s dis. – deficit of 10 c. f.) 4. Violationofthe last stage blood coagulation (afibrinogenemia)

  29. STATISTIC • Amount of coagulopathie’s types in general statistic: Haemophylia А68 – 78% Willebrand’s disease9 – 18 % Haemophylia В6 – 13 % Haemophylia С, parahaemophylia, hypoproconvertinemia1 – 2 % Other types are the medical casuistry (occur very reary)

  30. Haemophylia А Haemorrhag syndrome caused by hereditary deficit of 8 f. procoagulative unit Structure of 8 Factor(highmass protein) 1. Procoagulativeglucoprotein (VIII:C) 2. Glucoprotein, which causesthrombocytes adgesion (VIII:WF) 3. Glucoprotein, which causesthrombocytes adgesion due to ristomicine (VIII:R-cоf.) 4. Antigen marker of VIII:C(VIII:C АG) 5. Antigen marker of VIII:R-cоf (VIII:R АG) Activity of factors VIII:K and VШ:W is being decreased at the decrease of complex structure mass.

  31. Haemophylia А • ETHIOLOGY – gene anomaly in X-chromosomewhich controls synth. of procoagulativeglucoprotein (VIII:C) They ill – males (46, XhY) – females(46,XhXh ), (45,Xh O) Types; - Hemophilia А+(antigen positive hemophilia is the result of abnormal VIII:C factor synthesis), they ill 8 –10 % - HemophiliaА- (antigen negative hemophilia– no synthesis of VIII:C factor), they ill 90 –92 % Clinical signs:hemorrhages in major joints, deep hematomas of skin, intramuscular hematomas, massive and prolonged posttraumatic bleedings, intra ubdominal bleedings, in side gastro-intestinal tract

  32. Children of last Russian Car, his sun suffered from hemophilia A.

  33. HaemophyliaB • ETHIOLOGY – gene anomaly in X-chromosomewhich controls synth. of 9 coagul. f. They ill – males (46, XhY) – females(46,XhXh ), (45,Xh O) Types; - Hemophilia B+(antigen positive hemophilia is the result of abnormal 9 factor synthesis) - HemophiliaB- (antigen negative hemophilia– no synthesis of 9 factor) Clinical signs:hemorrhages in major joints, deep hematomas of skin, intramuscular hematomas, massive and prolonged posttraumatic bleedings, intra ubdominal bleedings, in side gastro-intestinal tract

  34. Hemophilia in newborn(subcutaneous hematoma) Subcutaneous hematoma in childe after injection

  35. Acquired coagulopathy • Character – polydeficit • ETHIOLOGY 1. immune inhibition of coagulative factor(antigenic noncompatibility of the mother and the fetus) 2. K-vitamin dependent coagulative factors deficit (7, 10, 9, 2) а) poor vitamin K synthesis(dysbacteriosis, profusal diarrhea, enteropathies) б) violation of vitamin K absorbtion(obturative icterus) в) damage of the liver 3. heparinor herudin overdose

  36. Hypercoagulation • Pathological property of the blood which is characterised by trombuses formation inside the vessels trombosis DIC-syndrome

  37. DIC-syndromedessiminative intravascular blood coagulation syndrome • CLASSIFICATION • By clinical course 1) aqute 2) chronic 1) local 2) diffuse

  38. ETHIOLOGY 1. Infection, sepsis(bacteriemia, virusemia) 2. Chok-traumatic, hemorrhagical, anaphylactic, cardiogenic, septic(at sepsic chok death occures in 100 % cases) 3. Surgical operation 4. Thermal burns and chemical ones 5. Terminal states(stages of the dying), heart stop 6. Acute intravessels hemolysis of the RBSs 7. Obstetrics pathology (20-25 %) 8. Leucaemias (33-45 %) 9. Immune diseases 10. Allergy reactions

  39. DIC-syndrome stages • 1) Hypercoagulation(numerous thrombuses formation because hyperactivity of coagulative system) • 2) Coagulopathy of using(exhaustion of coagulative system, overusing of the thrombocytes for thrombuses formation) • 3) Hypocoagulation(decrease of coagulative system activity, activation of anticoagulative system and fibrinolysis) • 4) Finishing(recovery, complications, death)

  40. DIC-syndrome pathogenesis 1) Hyperthrombinemia (thromboplastinfrom injured tissues and blood cells comes in to the bloob and conduces thrombin formating) At infectionactive monocytes start to produce such coagulative factors as7, 10, 9, 2

  41. DIC-syndrome pathogenesis 2) Thrombocytes aggregation (it causes thrombocytopenia of using which results in bleeding) 3) Erhythrocyte’s damage and hemolysis (couses АDP excretion, thrombocyte’s adgesion and aggregation)

  42. DIC-syndrome pathogenesis 4)“Humoral protease detonating” (activation of coagulative factors, anticoagulative factors, fibrinolytics, proteins of kallikrein-kinin system, proteins of complementsystemcouses accumulation of great amount of proteins disintegration metabolitesin the blood. They are very toxic and damage a vascular wall and tissues)

  43. DIC-syndrome pathogenesis 5) Blood coagulative factors exhaustion(causes bleeding) 6) Exhaustion of coagulative system(resultes inthrombosis)

  44. DIC-syndrome clinical signs 1. Hemocoagulative shock (at acute DIC-syndrome) reason microcirculation violations(causes tissue’s hypoxia) accumulation of proteolysis toxic substances signs * decrease of arterial pressure * decrease of central venous pressure * development of profusal bleeding(promotes the transformation of hemocoagulative shock into the hemorrhagic one)

  45. DIC-syndrome clinical signs 2. Нaemostasis violation 1. Hypercoagulation Main sign – thrombosis Blood is clottedin test-tube 2. Hypocoagulation Main sign –bleeding At the same time exhaustion of fibrinolysis system occures)

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