7b..bleeding disorder

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7b..bleeding disorder

  1. 1. BLEEDINGBLEEDINGDISORDERSDISORDERSDR.RAFI AHMED GHORIProfessorMedical Unit ILUMHS Jamshoro.
  2. 2. BLEEDING DISORDERBLEEDING DISORDERDefinition:Definition:Disorder characterized bySpontaneous bleeding.Excessive bleedingfollowing trauma.
  3. 3. EtiologyEtiologyVESSEL WALL ABNORMALITIES:PLATELETS DISORDER:COAGULATION DISORDER:
  4. 4. VESSEL WALL ABNORMALITIES:VESSEL WALL ABNORMALITIES:Vessel wall abnormalities may be congenital OR acquired i-evasculitis may result in purpuric lesions.CAUSES OF NON-THROMBOCYTOPENIC PURPURA: 1. Senile purpura 2. Fictitious purpura 3. Henoch-Schonlein purpura 4. Vasculitis 5. Paraprotienaemias 6. Purpura fulminans 7. Embolic purpura
  5. 5. VESSEL WALL ABNORMALITIES:VESSEL WALL ABNORMALITIES:HERIDITARY HEMORRHAGICTELANGECTSIASIS: Dominant inherited condition. There is a telengectiasis andsmall aneurysms found on finger tips, face, nasal passages,tongue and GIT. Small group of people develop pulmonary A/Vmalformation. Pt either develops recurrent bleeding/epistaxis/iron deff:anemia due to occult GIT bleeding.Rx.Rx. Iron therapy for blood loss. .Local cautery/laser therapy for single lesion from bleeding(epistaxis). Estrogens may be tried.
  6. 6. VESSEL WALL ABNORMALITIES:VESSEL WALL ABNORMALITIES:EHLERS DANLOS DISEASE:Congenital disorder of collagensynthesis in which capillaries are poorlysupported by s/c collagen andecchymosis are commonly observed.
  7. 7. PLATELETS DISORDER:PLATELETS DISORDER:  QUANTITATIVE PLATELETSDYSFUNCTIONQUALITATIVE PLETELETDISORDER:
  8. 8. PLATELETS DISORDER:PLATELETS DISORDER:QUANTITATIVE PLATELETSQUANTITATIVE PLATELETSDYSFUNCTIONDYSFUNCTION ((Thrombocytopenia) Thrombocytopenia) Mechanism: 1 Failure of megakaryocytic maturation. 2 Excessive platelets consumption after their release intocirculation i-e ITP, DIC etc. 3 Platelets sequestration in enlarged spleen i-eHYPERSPLEENISM.S/S:• Petechial cutaneous bleeding, intracranial bleeding andoozing from mucus membrane/surface.• Characterized by decreased platelets count and prolongbleeding time.
  9. 9. ((Thrombocytopenia)Thrombocytopenia) Causes:Causes:Marrow Disorder Aplastic anemia Hematologicmalignancy Myelodysplasticdisorder B12 deff. Chronic alcoholismNon Marrow DisorderImmune disordersITPDrug inducedSec: CLL, SLEPost transfusionDICTTPHU syndromeHyperspleenismSepsisHeamangiomasViral infectionManagement:• Rx Underlying cause• Platelet transfusion
  10. 10. IDIOPATHIC THROMBOCYTOPENICIDIOPATHIC THROMBOCYTOPENICPURPURA.PURPURA.Autoimmune antibody IgG is formed againstunknown antigen of platelets membrane/surface.Antipletelet antibody binds to complement, plateletsare not destroyed by direct lysis.Rather destruction takes place in spleen, wherespleenic macrophages with Fc bind to antibodycoated platelets.
  11. 11. IDIOPATHIC THROMBOCYTOPENICIDIOPATHIC THROMBOCYTOPENICPURPURA.PURPURA. (Clinical Features)(Clinical Features)In Children:Often precipitated by viral infection and usuallyself limited Asymptomatic not febrile. Present with mucosal/skin bleeding,mennorrhagia, purpura, petechiae.Adults:• Commonly affects female.• Ratio 2:1 (male/female ratio)• Peak incidence 20-50 years of age.
  12. 12. IDIOPATHIC THROMBOCYTOPENICIDIOPATHIC THROMBOCYTOPENICPURPURA.PURPURA.Δ LAB: platelets below 10,000 /ml. Bone marrow will appear normal.Rx PREDENISONONE: 1-2 mg/kg/day. SPLEENECTOMY: immunoglobulin 1g/kg/day 2-3 days. DANAZOLE: 600mg/day response rate is 50% IMMUNOSUPPERESSIVE DRUGS: i-e vincristine,vinblastine, azathioprine, cyclosprin, cyclophosphomide.Prognosis: The prognosis for remission is good. Disease is initiallycontrolled with prednisolone, spleenectomy is definite Rx.
  13. 13. EVANS SYNDROME:EVANS SYNDROME:ITP + Autoimmune hemolytic anemia10% cases.These pts shows spherocytosis,reticulocytosis + anemia.
  14. 14. THROMBOTIC THROMBOCYTOPENICTHROMBOTIC THROMBOCYTOPENICPURPURA:TTP:PURPURA:TTP:TIP is an uncommon syndrome withMicroangiopathic hemolytic anemia,Thrombocytopenia andMarkedly increased LDH,Non-infectious fever,Neurologic disorder,Renal abnormalities are less commonly seen.Pathogenesis may be diff: of von Willibrand’s diseasefactors clearing protease, in some case antibodydirected against protease.
  15. 15. TTP:TTP:Clinical features:20-25 yrSlightly common in female.AnemiaBleedingFeverNeurological symptomsHead acheConfusion aphasiaAltered consciousnessHemi paresis + seizures.
  16. 16. TTP:TTP:LAB:Blood CPAnemiaReticulocytosisCirculating nucleated cells.Microangiopathic pictureFragmented RBC’s i-e(schistocytes, helmetcells, triangle forms)Thrombocytopenia.Hemolysis, IncreasedIndirect bilirubinHemoglobinemiaMethem-albuminia.Increased LDH.Coomb’s test –ve.Coagulation test:Normal PT, APPTT,fibrinogen.Elevated. FDP(fibrindegradation product)may be
  17. 17. TTP:TTP:Rx:Plasmapheresis with /without prednisone, antiplatelets aspirin 325 mg/daily , dipyridamole 75mg × TDS may be given.Combination spleenectomy, steroids and dextranmay be used with success.Immuno suppressive therapy i-e(cyclophosphomide).Prognosis:80-90 % Pts recover completely with plasmapharesis while 20% pts will be chronic andrelapsing.
  18. 18. QUALITATIVE PLETELET DISORDERQUALITATIVE PLETELET DISORDERCONGENITAL:Glansmann’sthrombostheniaBernard souliarsyndromeStorage pool disease•ACQUIRED•Myeloproliferative disorder.•Uremia•Drugs i-e NSAIDS Aspirin•Autoantibody•Paraprotiens•Acquired storage pool disease•Fibrin degradation products•Von Willibrand’s disease
  19. 19. QUALITATIVE PLETELET DISORDERQUALITATIVE PLETELET DISORDERBERNARD SOULIER SYNDROME:BERNARD SOULIER SYNDROME: Autosomal recessive intrinsic platelets disorder. lack of glycoprotein (41 b) receptor for von Willibrand’sfactor.Clinical Features: Presents with mucosal bleeding and post operatively aswell.LAB: Thrombocytopenia may be present, and abnormally large. BT is prolonged Von Willibrand’s factor NormalRx: Platelet transfusion
  20. 20. QUALITATIVE PLETELET DISORDERQUALITATIVE PLETELET DISORDERGLANSMANN’s THROMBASTHENIA:GLANSMANN’s THROMBASTHENIA: Autosomal recessive disorder. Lack of receptors (containing glycoprotein II b + III a) forfibrinogen on platelets.Clinical Features: Mucosal bleedingLAB: Platelets no’s and morphology are normal B.T is prolonged Platelets fails to aggregate in respond to typical agent (ADP,collagen, thrombin) but aggregate in respond to risocetin.Rx: Platelet transfusion
  21. 21. QUALITATIVE PLETELET DISORDERQUALITATIVE PLETELET DISORDERVON-WILLIBRAND’S DISEASE:VON-WILLIBRAND’S DISEASE:Autosomal dominant and gene for (VWF) islocated on chromosome 12.VWF is synthesized by endothelial cells andmegakaryocyticIt acts as carrier protein for factor VIII which it isnon-covalently bound. A defect therefore leads todecreased plasma factor VIII level.It forms bridges b/w platelets and sub endotheliumeg collagen allowing platelets to adhere to damagedvessel walls. There fore defect of VWF leads toprolong bleeding after minor trauma.
  22. 22. QUALITATIVE PLETELET DISORDERQUALITATIVE PLETELET DISORDERVON-WILLIBRAND’S DISEASE:VON-WILLIBRAND’S DISEASE:Clinical Features: Mucosal bleeding as already discussed.LAB: Reduced level of VWF which often accomplished by sec:reduction in factor VIII and prolonged bleeding time(B.T)Rx: MILD HAEMORRHAGES:Desmopressin 0.3 μg/kg, after which VWF levels usuallyraise 3 in 30-90 minutes MASSIVE HAEMORRHAGES:
  23. 23. COAGULATION DISORDER:COAGULATION DISORDER:Coagulation factor disorder can either caneither arise from single factor usually“congenital deficiency” eg factor VIIIresulting in HAEMOPHILIA-A ormultiple factor which is acquired eg Sec:to liver disease or warfarin therapy.
  24. 24. HEAMOPHILIA – A (CLASSIC TRUEHAEMOPHILIA)HAEMOPHILLIA – B (CHRISTMASDISEASE)COAGULATION DISORDER:COAGULATION DISORDER:CONGENITAL BLEEDINGCONGENITAL BLEEDINGDISORDER:DISORDER:
  25. 25. HEAMOPHILIA – A (CLASSIC TRUEHEAMOPHILIA – A (CLASSIC TRUEHAEMOPHILIA)HAEMOPHILIA) X-linked disorder Due to defect of factor VIIIC/F: Bleeding occurs as bruising when babies are about 6 monthold.Trauma results in excessively bleeding. Recurrent bleeding hemorrhage at following sitesknee, elbow, ankle, and hip. Mucus membrane internal bleeding of mouth, lips, gums,brain and kidney Muscle haematoma esp. calf and Psoas muscleRx Factor VIII infusion
  26. 26. HAEMOPHILLIA – B (CHRISTMASHAEMOPHILLIA – B (CHRISTMASDISEASE)DISEASE) Due to deff: of factor IXS/S: Same in type ARx Factor IX infusionLONG TERM COMPLICATIONCOMPLICATION due to repeated hemorrhage: Arthropathy of large joints eg knee, elbow Muscle atrophy due to haematoma Mononeuropathy due to pressure of haematoma.COMPLICATION due to therapy Antifactor VIII antibody develops Virus transmission Hepatitis A-B-C-D + HIV
  27. 27. COAGULATION DISORDERCOAGULATION DISORDERACQUIRED BLEEDINGACQUIRED BLEEDINGDISORDERDISORDERDICLIVER DISEASERENAL DISEASE
  28. 28. DISSAMINATED INTRAVASCULARDISSAMINATED INTRAVASCULARCOAGULATIONCOAGULATION DIC is condition characterized by thrombosis withincirculation. DIC can be induced by variety of differentmechanism. Endothelial cell damage eg endotoxic in G –ve septicemiaresults in tissue factor release which in turn leads tocoagulation cascade through extrinsic pathway. The presence thromboplastin from damaged tissue,placenta, fat embolus/following brain injury may activatecoagulation This results in consumption of platelets and coagulationfactors which secondarily activation of fibrinolysis leadingto bleeding tendency.
  29. 29. DICDIC::CAUSESInfectious: E Coli Nessieria meningitis Strep pneumonia MalariaObstetric RPOC Abruptio placentae Amniotic fat embolisms Pre-eclampsiaCancer Lung Pancreas ProstateCLINICAL FEATURES:Bleeding, thrombosis, bleedingfar from common thanthrombosis.Subacute DIC:Occurs primarily in cancerouspts results in superficial + deepvenous thrombosis.Other Manifestation:High incidence of cardiorespiratory failure
  30. 30. DICDICLAB:ThrombocytopeniaProlong PTAPPTT may benormal/increasedLow fibrinogenIncreased level D-dimmer
  31. 31. Treatment of DICTreatment of DICRx Underlying cause.General Measures: Correction of dehydration Renal failure Acidosis and ShockReplacement: Platelets transfusion if platelets counts below 10,000μg/l Fibrinogen with cryoprecipitate to maintain plasmafibrinogen level above 150 mg/dl FFP When thrombosis i-e DVT, Pulmonary their give Heparin.
  32. 32. THANKYOU

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