Hemostastis Pada Anak

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Hemostastis Pada Anak

  1. 1. Hemostasis Disease In Children dr. Bertha
  2. 2. Hemostatic Mechanism • Vascular response • Plateletadhesion • Platelet aggregation • Clot formation • Clot stabilization • Limitation of clotting (antitrombil III, Protein C, Protein S, TFP1) • Re-establishment of vascular potency  Fibrinolusis & vascular healing
  3. 3. History • Site, severity,duration of bleeding • Age of the symtomp onset • Spontaneous or after trauma • Previous history or family historyof bleeding • Does bruising (memar) occur spontaneously? • IS there has been previous surgery or dental procedure? • Menstrual history
  4. 4. Physical examination • Symptoms primarily associated with mucous or skin (mucocutaneous bleeding)  defects in platelet or blood vessel wall interaction (vWF disease) • Or muscle & joints bleeding (deep bleeding)  clotting factor deficiency • Presence of petechiae, ecchymoses, hematomas, hemarthroses, or mucous bleeding
  5. 5. Trombin time vWF -Platelet count -Bleeding time -PT / aPTT Specific work-up
  6. 6. Laboratory • Bleeding time (BT) – Assesses platelet function & their interaction with vascular wall – Platelet < 100.000/µL  prolonged BT – Disproportionate BT  qualitative platelet defects or vWF disease • aPTT – Measures the initiation of clotting (intrinsic pathway) – doesn’t measure factor VII, XIII or anticoagulant • PT – Measures extrinsic pathway – Normal in defiencies offactor VIII, IX, XI or XIII
  7. 7. Laboratory • TT – Measures final step of the clotting cascade – Prolonged  reduced fibrinogen levels • Dysfunctional fibrinogen (hypo/afibrinogenemia) • Substances that interfere with fibrin polymerization (heparin or fibrin split products)  reptilase time • Mixing studies – if there is unexplained prolongation of PT, PTT or TT – Normal plasma + patient’s plasma  repeat lab exam • Correction of PT/PTT by mixing  clootting factor deficiencie • Not corrected + bleeding  inhibitor • Not corrected, no bleeding  lupus-like-anticoagulant • Clotting factor assays
  8. 8. Hemophilia • Hemophilia A  Factor VIII deficiencies (85%) • Hemophilia B  Factor IX deficiencies ( 10-15%) • Most common & serious congenital coagulation factor deficiencies • Prevalence 1:5000 males • No racial predilection • Clinical finding  same
  9. 9. Hemophilia • Classification – Severe deficiency  <1% factor activity • Spontaneous bleeding – Moderate deficiency  1-5% factor activity • Mild trauma to induce bleeding – Mild deficiency  >5% factor activity • May be asymptomatic, took years to diagnose
  10. 10. Hemophilia • Clot formation is delayed & fragile • When bleeding occurs in the closed space  tamponade • Open wound  profuse bleeding • Bleeding symptoms may be present in utero • Neonates  intracranial bleeding • Easy bruising, IM hematomas, hemarthroses • Bleeding from minor trauma of the mouth  persist for days
  11. 11. Hemophilia • Iliposoas bleeding  life threatening – Inability to extend the hip – Confirmed by UTZ or CT scan – Aggresive therapy • Life threathening bleeding – CNS, Upper airways bleeding – External bleeding – GI bleeding
  12. 12. Hemophilia • Prolonged PTT • Factor assay: – Severe def. – Moderate def. – Mild def. • Inhibitor assay
  13. 13. Treatment • Prevention of trauma • Phychosocial • Avoid aspirin & NSAID • Replacement therapy – Recombinant fact. VIII/IX – Cryoprecipitate/ cryosupernate • Joint bleeding: – Ice pack – Elevate the limb – Immobilization of the limb • Supportive therapy • multidiciplinary
  14. 14. Chronic Complication • Chronic joint destruction • Risk of transfusions associated disease • Development of inhibitor
  15. 15. Disseminated Intravascular Coagulation (DIC) • Consumptive coagulopathy • Consumption of clotting factors, platelets & anticoagulant protein • Widespread intavascular deposition of fibrin  tissue ischemic & necrosis, generalized hemorrhagic state, hemolytic anemia
  16. 16. DIC • Trigger factors: – Hypoxia – Acidosis – Tissue becrosis – Shock – Endothelial damage – Septic shock – Incompatible blood transfusion – Snake bite
  17. 17. DIC • Manifestations: – Bleeding from surgical incision/venipuncture (pungsi vena)petechiae, ecchymoses – Organ damage – Anemia microangiopathic hemolytic anemia
  18. 18. DIC • Labs: – Prolonged PT, PTT & TT – Thrombocytopenia – Hemolytic process on blood smear – FDP, d-dimer appear in blood
  19. 19. DIC • Treatment: – Treat the cause – Restore normal homeostasis • Correct shock, acidosis, hypoxia – Blood component transfusions • Platelet concentrate, cryoprecipitate, FFP – Heparin infusions • For acute promyelocytic leukemia • Not indicated for septic shock, snack bite, massive head injury, incompatible transfusions
  20. 20. Platelet
  21. 21. The characteristic of platelets • Size: 1-4 µm (younger platelets are larger) • Mean platelet volume (MPV) : 8,9 ± 1.5 µm3 • Number : 150.000 – 400.000 / mm • Distribution : 1/3 in the spleen, 2/3 in blood stream • Life span : 7-10 days Bleeding may occur because : • Reduce in number (thrombsytopenia) • Defective in function
  22. 22. Thrombocytopeni based on pletelet sized Macrothrombocytes (MPV ↑) Microthrombocytes (MPV ↓) • ITP or condition with increased • Wiskott –Aldrich syndrome platelet turnover (eg. DIC) • TAR syndrome • Bernard-Soulier Syndrome • Some storage pool diseases • May Heggin anomaly and other MYH-9-Related disease • Iron def. Anemia • Swiss Cheese platelet syndrome Normal size (MPV normal) • Disease with hypocellular marrow • Montreal platelet syndrome or infiltrated with malignant disease • Gray platelet syndorme • Various mucopolysaccharisoses
  23. 23. Clasification of Trombocytopenia Incrase platelet Disorder of platelet destruction distribution or pooling Thrombocyto penia Decrased platelet Pseudothrombocyto production penia
  24. 24. Clasification of Trombocytopenia Hypersplenism (Portal hypertension, Gaucher disease,cyanitic Disorder of congenital, heart disease, platelets neoplasm, infection) distribution or pooling Hypothermia
  25. 25. Clasification of Drugs: Chlorothiazide, ethanol Trombocytopenia Constitusional: Hyperplasia or Rubella,… suppresion of megakaryocyte Ineffective Thrombopoeisis: …….. Disorder of control Decrase platelet mechanism: production Trombopoetin deficiency Acquired myelositic disorder: Drugs Marrow Benign: Infiltrative Osteoporosis Process Malignancy
  26. 26. Clasification of Trombocytopenia Platelet inactivation during bloof collection Pseudo- thrombocytopenia Undercounting of megathrombocytes In vitro agglutination of platelets to EDTA
  27. 27. Clasification of Idiopathic (ITP) Trombocytopenia Secondary: Immune Infection, drugs, Thrombocytopenia SLE, etc Neonatal: Autoimune, Erito- Increase platelet blastosis fetalis destruction Platelet consumption Non-immune Thrombocytopenia Platelet destruction: drugs
  28. 28. Immune Thrombocytopenia The most frequent cause of thrombocytopenia is immune mediated platelet destruction due to: 1. autoantibodies 2. drug-dependent antibodies 3. alloantibodies
  29. 29. Immune (Idiopathic) Thrombocytopenic Pupura A syndrome characterized by thrombocytopenia : 1. Shortened platelet survival 2. Presence of antiplatelet antibody in the plasma 3. Increase megakaryocytes in the bone marrow
  30. 30. ITP • The syndrome can be: – Acute • Platelet count return to normal within 6 month & relaps does not occur • Most in children – Chronic • Platelet count remain low beyond 6 month • More common in adult – Recurrent • Platelet count decrease after having returned to normal
  31. 31. Predisposing Factor • 50-80% : infection (usually viral) prior to thrombocytopenia • About 20% : a specific infection can be identified, eg. Rubella, measel, varicella, pertussis, mumps, infectious mononucleosis, CMV, parvovirus or bacterial • Measel or smallpox vaccination
  32. 32. Clinical Manifestation • Skin: Ecchymosess/purpira usually on the anterior surface of lower extremities and body prominences (ribs, scapula, shoulders, legs, pubic) • Mucous membranes: subconjunctival, buccal mucosa, soft palate • Menorrhagia • Hematemesis & melena  infrequent • Others: nose, gum, G.I, Kidnets (usually at the onset of the disease
  33. 33. Clinical manifestation • Intracranial bleeding: – Usually preceded by: • Headache, dizziness, acute bleeding at other place • Retinal hemorrage • Middle ear  hearing impairment • Deep muscle hematoma and hemarthrosis • Rare, seen after i.m injection or significant trauma • Characteristic of plasma coagulation
  34. 34. Laboratory Findings • Low platelet count – Always <150.000 /mm3 – Often <20.000 /mm3 in patients with severe generalized hemorrhagic manifestations – MPV ( N : 8.9 + 1.5 um3) • Blood smear – Thormbocytopenia must be confirmed by peripheral blood examination to exclude the diagnosis pseudothrombocytopenia, the presence of megathrombocytes and other hematologic manifestation – Blood semar normal apart from thrombicytopenia • Anemia present in proportion to amount of blood loss
  35. 35. Bone Marrow Aspiration • Indication – Atypical presentation – Poor respone to therapy – To exclude other hematologic disorder sucg as leukemia • Characteristic – ↑ ,megakaryocytes, immature and asence of budding – Nomlar erythroid and myeloid cells – Occasionally eosinophilia – Erythroid hyperplasia if significant blood loss
  36. 36. Intracranial Hemorrhage • Incidence : 0,1 – 0,5 % • Age: 13 month – 16 years • Platelet count : – < 10.000 /mm3 in 73% cases – 10-20.000 /mm3 in 25% of cases – >20.000 /mm3 in 2% of cases • Interval between diagnosis of ITP and ICH: – <4 wekks in 51% of cases – 4 weeks – 9 years in 49%of cases (mean 27 weeks)
  37. 37. Intracranial Hemorrhage • Risk Factors in 45 % cases of ICH include: – Head injury (29%) – Aspirin treatment (5%) – AV malformation (17%) – Mucocutaneous hemorrhage (49%) • Site of ICH: – Intra cerebral (77%) – Subdural hematoma (23%) • 50 % had prior tretament with steroid and / or IVIG • 54% survival, most without permanent damage
  38. 38. Supportive Treatment • No treatment is required when platelet count >20.000 /mm3 , asymptomatic or has mild bruising but no evidence of mucous membrane bleeding • Competitive sport should be avoided • Depoprovera or any other long-acting progesteron in suspending menstruation for several month • Aspirin, Nonsteroidal antiinflammatory agents and any other drug the interfere with platelet function should not be given
  39. 39. Farmacological Treatment • Treatment choice : Steroid, IVIG, and anti–D • Indication – Platelet count <20.000 /mm3 and significant mucous membrane bleeding – Platelet <10.000 /mm3 and minor purpura
  40. 40. Steroid Therapy • Mechanisms: – Inhibits phagocytosis of antibody coated platelet in the spleen  prolongs platelet survival – Improves capillary resistance and thereby improve platelet economy • Dose and Duration: – Dose : 2mg/kg/day (max. 60/mg/day) in divided dose. Tap off in 5-7 day interval and stopped at the end of 21-28 days, regardless of the response – In severe cases methylprednisolone 30mg/kg/day (max 1 g/day) for 3 days • Prolonged case of steroid in undesirable: – Worsen the thrombocytopenia and depress platelet [rpduction – Side effect : weight gain, caushingoid facies, fluid retention, acne, hyperglycemia, hypertension, mood swings, pseudotumor cerebri, cataracts, growth retradation , avascular necrosis
  41. 41. IVIG • Mechanism of action – Reticuloendothelial Fc-receptor blockade – Activation of inhibitor pathways – Decrease autoantibody synthesis • Indication – Neonatal Symptomatic Immune Thrombocytopenia  Infant less than 2 y.o are generally more refractory to steroid treatment – Alternative therapy to corticosteroid therapy • Much more expensive and has significant side effects
  42. 42. Anti –D Therapy • Plasma derived gamma immune globulin of anti –Rh antigen • Mechanism action : – Blockade of Fc receptor of reticuloendothelial cell • Platelet is increase after 48 hours, therefore the therapy is not appropriate for emergency treatment • Patients who have not undergone splenectomy and Rh positive are more likely to respond to IV Anti-D
  43. 43. Splenectomy • Indication – Severe acute ITP with acute life-threatening bleeding and not responsive to medical treatment – Chronic ITP with bleeding symptom or platelet count persistently below 30.000 /mm3 an not responsive to medical treatment for several years – In very active patient subject to frequent trauma, early splenectomy may be indicated • Because the hazard of overwhelming postsplenectomy infection (OPSI) the procedure should be performed after clear indication
  44. 44. Splenectomy • Indication for splenectomy are rare because of judicious use if steroid and IVIG • It is rarely necessary to perform splenectomy before 2 years adter diagnosis • Laparoscopic splenectomy is preferable to open splenectomy • Up to 70% have complete and long-lasting recovery • 40% wuth persistent thrombocytopenia after splenectomy have acsseory spleen
  45. 45. Treatment Algorithm Yang skema itu.. Gak keliatan di foto.. Maaf ya kawan..
  46. 46. Live Threatening Hemorrahage • Platelet transfusion • Methylprednisolone 500 mg/m2 IV per day for 3 days • IVIG 2 /kg for 12 hours infusion • Emergency splenecomy
  47. 47. Prognosis • Excellent, 50 % recover within 1 month % 70-80% within 6 month • Spontaneous remission after 1 year in uncommon, but may occur even after several years • When demonstration underlying cause, the prognosis is related to the cause • Age older than 10 years, insidious onset, female are associated with chronic ITP • 50-60 % chronic ITP………….. without any other therapy and without splenectomy

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