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November 23, 2010

November 23, 2010. Idiopathic Throbocytopenic Purpura. Idiopathic Thrombocytopenic Purpura. Most common cause of isolated thrombocytopenia in otherwise healthy children Antiplatelet antibody Binds to platelet surface Destruction by phagocytosis (spleen, liver) 50% follow viral illness

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November 23, 2010

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  1. November 23, 2010

  2. Idiopathic Throbocytopenic Purpura

  3. Idiopathic Thrombocytopenic Purpura • Most common cause of isolated thrombocytopenia in otherwise healthy children • Antiplatelet antibody • Binds to platelet surface • Destruction by phagocytosis(spleen, liver) • 50% follow viral illness • Following live vaccines (MMR)

  4. Clinical Presentation • History/Physical • Sudden onset petechiae/bruising • Mucosal hemorrhage 30% • Severe bleeding in only 3% • Labs • Isolated thrombocytopenia • <20K (frequently <5K) • Normal Hgb, MCV, WBC, PT/PTT • Smear unremarkable • paucity of platelets (large platelets)

  5. Acute ITP • Resolves spontaneously within 6 months • Bleeding tendency lessens within 1-2wks

  6. Treatment • Avoidance of NSAIDS • Limit activities • No contact sports • Reassurance of parents

  7. Reassurance • Life-threatening hemorrhage very rare • Intracranial hemorrhage 0.5 to 0.1% • Young platelets • Large • Metabolically active • “Sticky” • Underestimated by cell counter

  8. Treatment • Prednisone • IVIG • Rho(D) Immune Globulin • Avoid platelet transfusion • Rapidly destroyed • Consider with life-threatening bleed

  9. Treatment: Steroids • Prednisone • 1-2 mg/kg/day • Decreases as prednisone tapered (2-3wks)

  10. Treatment: IVIG • Costly • More effective than steroids • 80-90% plts >20k within 3 days • Not shown to prevent serious bleeding • Side effects

  11. Treatment: Rho(D) Immune globulin • May be used if: • Hgb >10 • Rh + • Efficacy similar to that of IVIG • Hemolysis possible

  12. Chronic ITP

  13. Chronic ITP • >6 months duration • Generally benign • Not requiring aggressive therapy • Platelets 30-80k • Some bruising and petechiae

  14. Chronic ITP • Treatment with steroids, IVIG, Rho(D)IG • Transient increased platelets • Not curative • Useful in emergency or at surgery

  15. Chronic ITP • Most improve over time • Allow school and childhood activities • NO contact sports • Splenectomy in refractory cases • 60-80% cure rate

  16. Congenital Disorders

  17. Wiskott-Aldrich Syndrome • X-Linked • Recurrent infections • Otitis, Pneumonia • Thrombocytopenia • LOW MPV (mean plt volume) • Risk for malignancies • Treatment: B.M. Transplant

  18. Thrombocytopenia Absent Radius • WBC usually high • Normal Hgb • Symptomatic by 4 m/o

  19. Think about it… • Child with bleeding • Normal: • Plt count • Coags • GlanzmannThrombasthenia • Disorder of platelet Function • NSAIDS overdose • Abnormal function

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