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Bleeding Disorders By Dr. Sabir M. Ameen
Vasoconstriction
1° Hemostasis
2° Hemostasis
Hemostasis <ul><li>Lab Tests </li></ul><ul><li>CBC- Plt </li></ul><ul><li>BT ,(CT) </li></ul><ul><li>PT </li></ul><ul><li>...
History <ul><li>1. Site of bleeding: </li></ul><ul><li>* Muscle & joint bleeds indicate a coagulation defect </li></ul><ul...
History <ul><li>2. Duration: congenital or acquired </li></ul><ul><li>3. Precipitating cause: if spontaneous indicate seve...
History <ul><li>5. Family history:  </li></ul><ul><li>* Hereditary or acquired </li></ul><ul><li>* Negative history does n...
history <ul><li>7. Drugs: almost any drug can potentially produce bleeding( cytotoxics. NSAIDs…etc) </li></ul>
Examination <ul><li>Look for: 1. anemia: BM failure, leukemia </li></ul><ul><li>2. purpura, bruises, bleeding in mouth </l...
Clinical Features of Bleeding Disorders Platelet Coagulation  disorders fac disorders Site of bleeding Skin Deep in soft t...
Platelet  Coagulation Petechiae, Purpura   Hematoma, Joint bl.
Petechiae Do not blanch with pressure   (cf. angiomas) Not palpable   (cf. vasculitis) (typical of platelet disorders)
Hemarthrosis
Hematoma
Petechiae
Purpura
Ecchymosis
Screening tests <ul><li>1. PLT count: thrombocytopenia </li></ul><ul><li>2. Bleeding time( normal <8 min.): î in thrombocy...
Causes of bleeding <ul><li>1. Thrombocytopenia: </li></ul><ul><li>a- viral infections </li></ul><ul><li>b- drug-induced </...
Causes..cont. <ul><li>2. Clotting factor deficiency: </li></ul><ul><li>a- liver disease </li></ul><ul><li>b- drugs: warfar...
Causes…cont. <ul><li>3. CT atrophy:  </li></ul><ul><li>a- old age </li></ul><ul><li>b- steroid therapy </li></ul><ul><li>c...
Idiopathic thrombocytopenic purpura(ITP) <ul><li>It is due to auto- antibodies directed against PLT membrane glycoprotein ...
ITP <ul><li>C/F: 1. in children: sudden onset of purpura, oral or nasal bleeding, usually 2-3 wk after a viral illness </l...
ITP <ul><li>LAB. </li></ul><ul><li>1. CBC: reduced PLT count </li></ul><ul><li>2. BM: increased megakaryocytes </li></ul>
management <ul><li>1. In children: usually it is self-limiting, if severe purpura or epistaxis, or PLT <10000 give steroid...
management <ul><li>2. In adults:  prednisolone  1 mg/kg/d for 3-4 wk then gradually tapered over 6-8 wk, relapse may occur...
management <ul><li>In severe cases iv  methylpredinsolone  with or without IVIg may be given </li></ul><ul><li>If still no...
Hemophilia A <ul><li>Factor VIII is synthesized mainly by liver , but also by spleen, kidney, and placenta, carried by vWF...
Hemophilia A <ul><li>C/F </li></ul><ul><li>At around 6 mon. child develop bruises and hemarthrosis as he starts to move ar...
Hemophilia A <ul><li>2. Moderate : 1-5% F VIII or IX: mild trauma or surgery causes hematoma </li></ul><ul><li>3. mild: 6-...
Hemophilia A <ul><li>With recurrent bleeding there will be synovial hypertrophy, destruction of cartilage and secondary os...
Shortening of Achilles tendon
Dosing guidelines for hemophilia A <ul><li>Mild bleeding :  </li></ul><ul><ul><li>Target: 30% dosing q8-12h; 1-2 days (15U...
Von Willebrand’s disease <ul><li>Usually it is a mild bleeding disorder of many types, the commonest being type I which is...
vW Dis. <ul><li>C/F </li></ul><ul><li>Bruising, epistaxis, menorrhagia, GI bleeding </li></ul><ul><li>LAB </li></ul><ul><l...
Treatment of von Willebrand Disease <ul><li>Cryoprecipitate  </li></ul><ul><ul><li>Source of fibrinogen, factor VIII and V...
Common clinical conditions associated with DIC <ul><li>Sepsis </li></ul><ul><li>Trauma :  Head injury ,  Fat embolism </li...
Pathogenesis of DIC Coagulation Fibrinolysis Fibrinogen Fibrin Monomers Fibrin Clot (intravascular) Fibrin(ogen) Degradati...
DIC Treatment approaches <ul><li>Treatment of underlying disorder </li></ul><ul><li>Anticoagulation with heparin </li></ul...
Management of Hemostatic Defects in Liver Disease <ul><li>Treatment for prolonged PT/PT  </li></ul><ul><ul><li>Vitamin K 1...
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Medicine 5th year, 10th lecture (Dr. Sabir)

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Transcript of "Medicine 5th year, 10th lecture (Dr. Sabir)"

  1. 1. Bleeding Disorders By Dr. Sabir M. Ameen
  2. 2. Vasoconstriction
  3. 3. 1° Hemostasis
  4. 4. 2° Hemostasis
  5. 5. Hemostasis <ul><li>Lab Tests </li></ul><ul><li>CBC- Plt </li></ul><ul><li>BT ,(CT) </li></ul><ul><li>PT </li></ul><ul><li>PTT </li></ul>Plt Study Morphology Function Antibody BV Injury Platelet Aggregation Platelet Activation Blood Vessel Constriction Coagulation Cascade Stable Hemostatic Plug Fibrin formation Reduced Blood flow Tissue Factor Primary hemostatic plug Neural
  6. 6. History <ul><li>1. Site of bleeding: </li></ul><ul><li>* Muscle & joint bleeds indicate a coagulation defect </li></ul><ul><li>* purpura, prolonged bleeding from superficial cuts, epistaxis, GI bleeding, menorrhagia, indicate PLT disorder, thrombocytopenia or vW disease </li></ul>
  7. 7. History <ul><li>2. Duration: congenital or acquired </li></ul><ul><li>3. Precipitating cause: if spontaneous indicate severe defect </li></ul><ul><li>4. surgery: dental extraction, tonsillectomy, circumcision. Bleeding immediately after surgery indicate defective PLT plug formation. Bleeding after some hours indicate failure of PLT plug stabilisation by fibrin due to coagulation defect. </li></ul>
  8. 8. History <ul><li>5. Family history: </li></ul><ul><li>* Hereditary or acquired </li></ul><ul><li>* Negative history does not exclude a hereditary cause, as e.g: about 1/3 of cases of hemophilia have negative family history (mutations). </li></ul><ul><li>6. Systemic disease: </li></ul><ul><li>Hepatic or renal failure </li></ul><ul><li>CT disease </li></ul>
  9. 9. history <ul><li>7. Drugs: almost any drug can potentially produce bleeding( cytotoxics. NSAIDs…etc) </li></ul>
  10. 10. Examination <ul><li>Look for: 1. anemia: BM failure, leukemia </li></ul><ul><li>2. purpura, bruises, bleeding in mouth </li></ul><ul><li>3. Telangiectasia of lips (HHT) </li></ul><ul><li>4. LN enlargement: leukemia, viral ( ITP) </li></ul><ul><li>5. Stigmata of chronic liver disease: spider nevi, clubbing, palmar erythema…etc </li></ul><ul><li>6. Fundal examination </li></ul>
  11. 11. Clinical Features of Bleeding Disorders Platelet Coagulation disorders fac disorders Site of bleeding Skin Deep in soft tissues Mucous membranes (joints, musc) (epistaxis, gum, vaginal, GI tract) Petechiae Yes No Ecchymoses (“bruises”) Small, superficial Large, deep Hemarthrosis / muscle bleeding Extremely rare Common Bleeding after cuts & scratches Yes No Bleeding after surgery or trauma Immediate, Delayed1-2 d usually mild often severe
  12. 12. Platelet Coagulation Petechiae, Purpura Hematoma, Joint bl.
  13. 13. Petechiae Do not blanch with pressure (cf. angiomas) Not palpable (cf. vasculitis) (typical of platelet disorders)
  14. 14. Hemarthrosis
  15. 15. Hematoma
  16. 16. Petechiae
  17. 17. Purpura
  18. 18. Ecchymosis
  19. 19. Screening tests <ul><li>1. PLT count: thrombocytopenia </li></ul><ul><li>2. Bleeding time( normal <8 min.): î in thrombocytopenia, PLT dysfunction, decreased vWF, vascular abnormality </li></ul><ul><li>3. PT: factors II, V, VII, X deficiency </li></ul><ul><li>4. PTT: factors II, V, VIII, IX, X, XI, XII deficiency, heparin therapy, Abs against clotting factors, lupus anticoagulant </li></ul><ul><li>5. fibrinogen: hypofibrinogenemia </li></ul>
  20. 20. Causes of bleeding <ul><li>1. Thrombocytopenia: </li></ul><ul><li>a- viral infections </li></ul><ul><li>b- drug-induced </li></ul><ul><li>c- B12 or folate deficiency </li></ul><ul><li>d- ITP, DIC, TTP/HUS ( consumption) </li></ul><ul><li>e- BM infiltration: leukemia, MM, Ca, myelofibrosis </li></ul>
  21. 21. Causes..cont. <ul><li>2. Clotting factor deficiency: </li></ul><ul><li>a- liver disease </li></ul><ul><li>b- drugs: warfarin, heparin </li></ul><ul><li>c- consumption: DIC </li></ul><ul><li>d- dilution: massive blood transfusion </li></ul><ul><li>e- congenital: hemophilia..etc </li></ul><ul><li>f- vit K deficiency: e.g, malabsorption </li></ul>
  22. 22. Causes…cont. <ul><li>3. CT atrophy: </li></ul><ul><li>a- old age </li></ul><ul><li>b- steroid therapy </li></ul><ul><li>c- wasting </li></ul><ul><li>4. Vessel wall disorders: </li></ul><ul><li>A- aspirin </li></ul><ul><li>B- Osler-Weber Rendu disease </li></ul><ul><li>C- angiodysplasia </li></ul>
  23. 23. Idiopathic thrombocytopenic purpura(ITP) <ul><li>It is due to auto- antibodies directed against PLT membrane glycoprotein IIb-IIIa which causes premature removal of PLTs by the monocyte-macrophage system </li></ul>
  24. 24. ITP <ul><li>C/F: 1. in children: sudden onset of purpura, oral or nasal bleeding, usually 2-3 wk after a viral illness </li></ul><ul><li>2. In adults: affects females more, with insidious onset, usually not associated with viral infection, but may be associated with CT disease. </li></ul><ul><li>It is characterised by remission and relapse. </li></ul>
  25. 25. ITP <ul><li>LAB. </li></ul><ul><li>1. CBC: reduced PLT count </li></ul><ul><li>2. BM: increased megakaryocytes </li></ul>
  26. 26. management <ul><li>1. In children: usually it is self-limiting, if severe purpura or epistaxis, or PLT <10000 give steroids ( prednisolone 2mg/kg/d) </li></ul><ul><li>If pesistent epistaxis GI bleeding, retinal hemorrhage : give PLT transfusion and IV IgG </li></ul>
  27. 27. management <ul><li>2. In adults: prednisolone 1 mg/kg/d for 3-4 wk then gradually tapered over 6-8 wk, relapse may occur on tapering </li></ul><ul><li>PLT transfusion and IVIg indicated in life-threatening bleeding. If the patient has two relapses , splenectomy is indicated, which is curative in 70% of patients, in the remainder the aim is to keep the patient free of symptoms rather than to raise level of PLT( e.g 5mg/d of prednisolone may be sufficient) </li></ul>
  28. 28. management <ul><li>In severe cases iv methylpredinsolone with or without IVIg may be given </li></ul><ul><li>If still not controlled give immunosuppressive drugs e.g: vincristine, cyclophosphamide </li></ul>
  29. 29. Hemophilia A <ul><li>Factor VIII is synthesized mainly by liver , but also by spleen, kidney, and placenta, carried by vWF, half-life in plasma is 12 hr. </li></ul><ul><li>It is sex-linked recessive and affects about 1/10000, thus all daughters of hemophiliacs are carriers and 50% of sisters are carriers. If a carrier has a son, he has 50% chance of having hemophilia and a daughter has 50% chance of being a carrier </li></ul>
  30. 30. Hemophilia A <ul><li>C/F </li></ul><ul><li>At around 6 mon. child develop bruises and hemarthrosis as he starts to move around. </li></ul><ul><li>Normal level of factor VIII is 50%-150%, and severity is measured according to this level: </li></ul><ul><li>1. severe: <1% F VIII or IX: liable for spontaneous hemarthrosis & muscle hematoma </li></ul>
  31. 31. Hemophilia A <ul><li>2. Moderate : 1-5% F VIII or IX: mild trauma or surgery causes hematoma </li></ul><ul><li>3. mild: 6-50% F VIII or IX: major surgery or injury results in excess bleeding. </li></ul><ul><li>Joints commonly affected include: knees, elbows, ankles, and hips. </li></ul><ul><li>They look hot, swollen, and very painfull and tender. </li></ul>
  32. 32. Hemophilia A <ul><li>With recurrent bleeding there will be synovial hypertrophy, destruction of cartilage and secondary osteoarthritis, </li></ul><ul><li>In muscles : calf, psoas: bleeding lead to ischemia, necrosis, fibrosis which will lead to contracture & shortening of tendons e.g achilles tendon making walking difficult. </li></ul>
  33. 33. Shortening of Achilles tendon
  34. 34. Dosing guidelines for hemophilia A <ul><li>Mild bleeding : </li></ul><ul><ul><li>Target: 30% dosing q8-12h; 1-2 days (15U/kg) </li></ul></ul><ul><ul><li>Hemarthrosis, oropharyngeal or dental, epistaxis, hematuria </li></ul></ul><ul><li>Major bleeding: </li></ul><ul><ul><li>Target: 80-100% q8-12h; 7-14 days (50U/kg) </li></ul></ul><ul><ul><li>CNS trauma, hemorrhage, lumbar puncture </li></ul></ul><ul><ul><li>Surgery </li></ul></ul><ul><ul><li>Retroperitoneal hemorrhage </li></ul></ul><ul><ul><li>GI bleeding </li></ul></ul><ul><li>Adjunctive therapy : </li></ul><ul><ul><li> -aminocaproic acid or DDAVP (for mild disease only) </li></ul></ul>
  35. 35. Von Willebrand’s disease <ul><li>Usually it is a mild bleeding disorder of many types, the commonest being type I which is autosomal dominant. </li></ul><ul><li>vWF is synthesized by endothelial cells and megakaryocytes and has two functions: 1. carrier for F VIII and 2. form bridges between PLT and subendothelial collagen </li></ul>
  36. 36. vW Dis. <ul><li>C/F </li></ul><ul><li>Bruising, epistaxis, menorrhagia, GI bleeding </li></ul><ul><li>LAB </li></ul><ul><li>* Decreased level of vWF, increased bleeding time, increased PTT </li></ul>
  37. 37. Treatment of von Willebrand Disease <ul><li>Cryoprecipitate </li></ul><ul><ul><li>Source of fibrinogen, factor VIII and VWF </li></ul></ul><ul><ul><li>Only plasma fraction that consistently contains VWF multimers </li></ul></ul><ul><li>DDAVP (deamino-8-arginine vasopressin) </li></ul><ul><ul><li> plasma VWF levels by stimulating secretion from endothelium </li></ul></ul><ul><ul><li>Duration of response is variable </li></ul></ul><ul><ul><li>Not generally used in type 2 disease </li></ul></ul><ul><ul><li>Dosage 0.3 µg/kg q 12 hr IV </li></ul></ul><ul><li>Factor VIII concentrate (Intermediate purity) </li></ul><ul><ul><li>Virally inactivated product </li></ul></ul>
  38. 38. Common clinical conditions associated with DIC <ul><li>Sepsis </li></ul><ul><li>Trauma : Head injury , Fat embolism </li></ul><ul><li>Malignancy </li></ul><ul><li>Obstetrical complications : </li></ul><ul><ul><li>Amniotic fluid embolism </li></ul></ul><ul><ul><li>Abruptio placentae </li></ul></ul><ul><li>Vascular disorders </li></ul><ul><li>Reaction to toxin (e.g. snake venom, drugs) </li></ul><ul><li>Immunologic disorders </li></ul><ul><ul><li>Severe allergic reaction </li></ul></ul><ul><ul><li>Transplant rejection </li></ul></ul><ul><li>Activation of both coagulation and fibrinolysis </li></ul><ul><ul><li>Triggered by </li></ul></ul>
  39. 39. Pathogenesis of DIC Coagulation Fibrinolysis Fibrinogen Fibrin Monomers Fibrin Clot (intravascular) Fibrin(ogen) Degradation Products Plasmin Thrombin Plasmin Release of thromboplastic material into circulation Consumption of coagulation factors; presence of FDPs  aPTT  PT  TT  Fibrinogen Presence of plasmin  FDP Intravascular clot  Platelets Schistocytes
  40. 40. DIC Treatment approaches <ul><li>Treatment of underlying disorder </li></ul><ul><li>Anticoagulation with heparin </li></ul><ul><li>Platelet transfusion </li></ul><ul><li>Fresh frozen plasma </li></ul><ul><li>Coagulation inhibitor concentrate (ATIII) </li></ul>
  41. 41. Management of Hemostatic Defects in Liver Disease <ul><li>Treatment for prolonged PT/PT </li></ul><ul><ul><li>Vitamin K 10 mg x 3 days - usually ineffective </li></ul></ul><ul><ul><li>Fresh-frozen plasma infusion (1200-1500 ml) </li></ul></ul><ul><ul><li>immediate but temporary effect </li></ul></ul><ul><li>Treatment for low fibrinogen </li></ul><ul><ul><li>Cryoprecipitate (1 unit/10kg body weight) </li></ul></ul><ul><li>Treatment for DIC (↑ D-dimer, ↓ factor VIII, thrombocytopenia </li></ul><ul><ul><li>Replacement therapy </li></ul></ul>
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