Bleeding and clotting disorders in children

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Bleeding and clotting disorders in children

  1. 1. Bleeding and coagulation disorders in children Dr.K.V.Giridhar Associate Prof. of Pediatrics GMC. Ananthapuramu, A.P., India. 4/16/2014 1
  2. 2. Definition: • Spontaneous bleeding. • Excessive & Prollanged bleeding following trauma. • Petechiae, purpura, echymosis, bruises, hematoma, hemarthrosis, IC bleeds, occult GI bleeds, melena, epistaxis.
  3. 3. Bleeding disorders Coagulation disorders
  4. 4. Hemostasis Tissue phase Platelet phase Clotting Phase Unstable clot Phase Clot stabilization Phase Clot retraction Phase Clot resolving Phase
  5. 5. THE CLOTTING MECHANISM INTRINSIC EXTRINSIC PROTHROMBIN(II) THROMBIN(III) FIBRINOGEN FIBRIN (I) V X Tissue ThromboplastinCollagen VIIXII XI IX VIII VII PT PTT Vit.K, Live r
  6. 6. Etiology • VESSEL WALL ABNORMALITIES: • PLATELETS DISORDER: • COAGULATION DISORDER:
  7. 7. Vessel Abnormalities • increased vascular fragility • manifest by petechial hemorrhages of skin/mucous membranes • not life threatening bleeding 1. congenital: a. Ehlers-Danlos syndrome (AD) b. hereditary hemorrhagic telangiectasia (AD) 2. acquired: a. hypersensitive vasculitis (i) drug reaction : immune complex deposit in vessel walls(Thaizide diuetics) (ii) Henoch-Schonlein purpura b. scurvy (vit C deficiency) Lab: BT, Plt count, PT, PTT will be normal
  8. 8. VESSEL WALL ABNORMALITIES: EHLERS DANLOS DISEASE: • Congenital disorder of collagen synthesis • in which capillaries are poorly supported by s/c collagen • ecchymosis are commonly observed.
  9. 9. VESSEL WALL ABNORMALITIES: HERIDITARY HEMORHAGIC TELANGECTSIASIS • Dominant inherited condition. • Telengectias, are small aneurysms found on finger tips, face, nasal passages, tongue and GIT. • few people develop pulmonary A/V malformation. • Pt. develops recurrent bleeding/epistaxis/ occult GIT bleeding, leads to Iron def. anemia Rx. • Iron therapy for blood loss. • Local cautery/laser therapy for single lesion • Estrogens may be tried.
  10. 10. Henoch-Schonlein purpura (nonthrombocytopenic) • generalized hypersensitivity vasculitis • uncertain cause(immune mediated) clinical Sx: • Purpura(palpable) • colicky abdominal pain • polyarthralgia • acute glomerulonephritis Rx: Prednisolone for2-4 weeks
  11. 11. PLATELETS DISORDER: • QUANTITATIVE PLATELETS DISORDERs • QUALITATIVE PLATELET DYSFUNCTION :
  12. 12. QUANTITATIVE PLATELETS DISORDER (Thrombocytopenia) Mechanisms: 1 Failure of megakaryocytic maturation. 2 Excessive platelets consumption after their release into circulation i.e ITP, DIC etc. 3 Platelets sequestration in enlarged spleen i.e HYPERSPLEENISM. S/S: · Petechial cutaneous bleeding, intracranial bleeding and oozing from mucus membrane & skin surface. · Lab: decreased platelets count and prolong bleeding time.
  13. 13. (Thrombocytopenia) Causes: Marrow Disorder Aplastic anemia Hem. malignancy Myelodysplastic disorder B12 deff. Non Marrow Disorder Immune disorders ITP, Drug induced Sec: ALL, SLE Post transfusion DIC, TTP HU syndrome, Hyperspleenism Heamangiomas Sepsis Viral infection Management: Rx Underlying cause Platelet transfusion
  14. 14. IDIOPATHIC THROMBOCYTOPENIC PURPURA(ITP) • An autoimmune antibody IgG is formed against unknown antigen of platelets membrane/surface. • Antipletelet antibody binds to complement, but platelets are not destroyed by direct lysis. • Destruction takes place in spleen, where spleenic macrophages destroyes antibody coated platelets.
  15. 15. IDIOPATHIC THROMBOCYTOPENIC PURPURA. (Clinical Features) In Children(acute): Often precipitated by viral infection and usually self limited  Asymptomatic not febrile.  Present with mucosal/skin bleeding, mennorrhagia, purpura, petechiae . Adults(chronic):  Commonly affects female.  Ratio 2:1 (male/female ratio)
  16. 16. IDIOPATHIC THROMBOCYTOPENIC PURPURA. Δ LAB: platelets below 10,000 /ml. Bone marrow will appear normal. Rx PREDNISONONE: 1-2 mg/kg/day. Immunoglobulin: 1g/kg/day 2-3 days. DANAZOLE: 600mg/day response rate is 50% IMMUNOSUPPERESSIVE DRUGS: i-e vincristine, vinblastine, azathioprine, cyclosprin, cyclophosphomide. SPLEENECTOMY: Prognosis: The prognosis will be good, if disease is initially controlled with prednisolone, spleenectomy is definite Rx.
  17. 17. EVANS SYNDROME: • ITP + Autoimmune hemolytic anemia. • These pts shows spherocytosis, reticulocytosis + anemia.
  18. 18. QUALITATIVE PLATELET DISORDER CONGENITAL: Glanzmann‟s thrombosthenia Bernard souliar syndrome Von Willibrand‟s disease ACQUIRED Myeloproliferative disorder. Uremia Drugs i-e NSAIDS Aspirin Autoantibody Fibrin degradation products
  19. 19. QUALITATIVE PLATELET DISORDER BERNARD SOULIER SYNDROME: Autosomal recessive intrinsic platelets disorder. Due to lack of glycoprotein (Gp1b), receptor for vonWillibrand‟s factor. Clinical Features: Presents with mucosal bleeding and post operative oozes. LAB: Thrombocytopenia may be present, and Plt.s are abnormally large in size. BT is prolonged Von Willibrand’s factor Normal Rx: Platelet transfusion
  20. 20. QUALITATIVE PLATELET DISORDER GLANZMANN’s THROMBASTHENIA: Autosomal recessive disorder. Lack of receptors (glycoprotein Ib & IIIa) for fibrinogen on platelets. Platelets fails to aggregate in respons to ADP, collagen, thrombin. Clinical Features: Mucosal bleeding LAB: Platelets no‟s and morphology are normal B.T is prolonged Rx: Platelet transfusion
  21. 21. QUALITATIVE PLATELET DISORDER VON-WILLIBRAND’S DISEASE: • Autosomal dominant(gene for vWF is located on chromosome 12.) • vWF is synthesized by endothelial cells and megakaryocytes • It acts as carrier protein for factor VIII by non-covalent bond. A defect therefore leads to decreased plasma factor VIII level. • It also forms bridges b/w platelets and sub endothelium. • There fore defect of vWF leads to prolonged bleeding.
  22. 22. VON-WILLIBRAND’S DISEASE: Clinical Features: • Mucosal bleeding (mild-massive) 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 usually raise 3 in 30-90 minutes • MASSIVE HAEMORRHAGES: Factor VIII
  23. 23. COAGULATION DISORDER: Coagulation factor disorder can either due to single factor def., i.e. a “congenitaldeficiency”, eg factor VIII resulting in HAEMOPHILIA-A or due to multiple factor def., which is an „‟acquired” eg Sec: to liver disease or warfarin therapy.
  24. 24. • HEAMOPHILIA – A (CLASSIC TRUE HAEMOPHILIA) • HAEMOPHILLIA – B (CHRISTMAS DISEASE). • X-linked recessive Inheritance. COAGULATION DISORDER: CONGENITAL BLEEDING DISORDER:
  25. 25. HEAMOPHILIA – A (CLASSIC TRUE HAEMOPHILIA) • X-linked disorder • Due to deff. of factor VIII C/F: • Bleeding occurs as bruising at the age of 6 months. • Trauma results in excessively bleeding. • Recurrent bleeding /hemorrhage in knee, elbow, ankle, and hip. (Hemarthrosis) • Mucus membrane /internal bleeding of mouth, lips, gums, brain and kidney also occur • Muscle haematoma esp. calf and Psoas muscle Rx • Factor VIII infusion
  26. 26. Hemophilia A
  27. 27. HAEMOPHILLIA – B (CHRISTMAS DISEASE) • Due to deff: of factor IX S/S: • Same in type A Rx • Factor IX infusion LONG TERM COMPLICATION COMPLICATION 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
  28. 28. COAGULATION DISORDER ACQUIRED BLEEDING DISORDER • DIC • LIVER DISEASE • RENAL DISEASE
  29. 29. DISSAMINATED INTRAVASCULAR COAGULATION • DIC is condition characterized by thrombosis within circulation. • DIC can be induced by different mechanisms. • Due to Endothelial cell damage by endotoxins in G –ve septicemia results in tissue factor release which in turn leads to coagulation cascade activation through extrinsic pathway. • The presence thromboplastin from damaged tissue, placenta & fat embolus (following brain injury & Fractures) may activate coagulation • This results in excessive consumption of platelets and coagulation factors, with secondary activation of fibrinolysis leading to bleeding tendency.
  30. 30. DIC: CAUSES Infectious: • E Coli • Nessieria meningitis • Strep pneumonia • Malaria Cancer • Lung,Pancreas, • Prostate CLINICAL FEATURES: Bleeding & thrombosis, bleeding is more than thrombosis. Subacute DIC: Occurs primarily in cancerous pts results in superficial + deep venous thrombosis. Other Manifestation: High incidence of cardio respiratory failure
  31. 31. DIC LAB: • Thrombocytopenia • Prolong PT • APTT may be normal/increased • Low fibrinogen • Increased level FDP/D-dimmer
  32. 32. Treatment of DIC Rx. Underlying cause. General Measures: • Correction of dehydration • Renal failure • Acidosis and • Shock Replacement: • Platelets transfusion if platelets counts below 10,000/l • cryoprecipitate to maintain plasma fibrinogen level above 150 mg/dl • FFP • Heparin, if there is DVT, Pulmonarythrombosis.
  33. 33. Approach to a child with bleeding disorder Bleeding Not sick sick Superficial bleeds Deep Bleeds CBC, BT Factor assay, Gene analysis Bone marrow Blood culture CBC, Bonemarrow LFT RFT FDP
  34. 34. THANKYOU

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