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Thrombocytopenia

Thrombocytopenia. ITP. Platelets. Platelets are an important component in the first phase of hemostasis - platelet plug formation When platelets are reduced in number or do not function normally, bleeding may occur. Platelets. The types of bleeding seen in platelet disorders include:

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Thrombocytopenia

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  1. Thrombocytopenia ITP

  2. Platelets • Platelets are an important component in the first phase of hemostasis - platelet plug formation • When platelets are reduced in number or do not function normally, bleeding may occur

  3. Platelets • The types of bleeding seen in platelet disorders include: • Skin and mucous membranes • Petechiae and purpura • Epistaxis, hematuria, menorrhagia • GI bleeding • Rarely ICH

  4. Platelets • Normal number: 150,000 - 400,000 • Distribution: 1/3 in spleen, 2/3 in PBL • Life Span: 7 - 10 days

  5. Causes of Thrombocytopenia • Decreased production • Increased destruction

  6. Decreased Production • Congenital • TAR syndrome • Fanconi’s anemia • Amegakaryocytic thrombocytopenia

  7. Decreased Production • Acquired • Infiltrative process • ALL, NBL etc • Aplastic anemia • Nutritional deficiencies • Drugs

  8. Increased Platelet Destruction • Immune • Neonatal alloimmune Thrombocytopenia • Drug related • HIV • Post-transfusion purpura • autoimmue • SLE • ITP

  9. Non-immune • Hemolytic-Uremic Syndrome • DIC • Cyanotic Heart Disease

  10. Qualitative Platelet Disorder • Wiskott Aldrich Syndrome • Bernard - Soulier Syndrome • May - Hegglin Anomaly

  11. Sequestration • Kasabach - Merrit Syndrome • Hypersplenism

  12. ITP

  13. ITP • Incidence: 1: 10,000 • Etiology: IgG coats platelets which causes destruction in spleen • Platelet survival is hours to min • There is an increased incidence of HLA B8 and B12 in patients with ITP

  14. Clinical Presentation • Onset (often abrupt) of petechiae, purpura, and easy bruisabiltiy • Platelet count low (Usually less 20,000) with normal WBC and diff and Hb/Hct • No significant lymphadenopathy or splenomegly • Often follows viral illness by 2 - 3 weeks or live virus immunization

  15. Differential Diagnosis • Drug induced thrombocytopenia • Viral related • HUS • Wiskott-Aldrich Syndrome • Infiltrative disorders

  16. History • Preceding viral infection • Risk factors for HIV • Onset of bruisabiltiy (abrupt Vs insidious) • Drugs being taken • Use of anti-platelet drugs • FHx of possible coagulation disorders

  17. History • Bone pain • Headache or Neurological changes • joint Sx • Previous Hx of low platelets or family history of low platelets

  18. Physical Exam • Location and types of bleeding • Fundiscopic and Neuro exam • Lymphadenopathy and/or splenomegly • Joint and skin exam

  19. Laboratory Data • CBC with diff and review of PBL smear • DAT • HIV* • ANA* • Chem* • BMA* • indicated if Dx of ITP in doubt or steroids to be used

  20. Outcome • 80 -- 90 % of patients will have spontaneous resolution within 6 months • 60 % will have resolution within 1 mo • Risk of life-threatening bleeding is < 1% • Treatment has not been shown to effect outcome

  21. Treatment • For children with platelet counts > 20,000 and no life-threatening bleeding, no therapy is indicated and there is no need for hospitalization • For children with platelet counts < 20,000: • Observation only • Treatment

  22. Treatment Options • IV IgG • Steroids • IV anti RhD (Winrho) • Splenectomy • Other

  23. IV IgG • IV IgG

  24. IV IgG • Blocks reticuloendothelial Fc receptor in the spleen and therefore there is no place for the platelet-antibody to be taken up • As IV IgG is degraded, the receptor sites become available and platelet destruction will recur • IV IgG has no effect on natural history of ITP

  25. IV IgG • 80 % of patients will respond • Response is usually rapid • Dose 1 gm/kg times 2 • Side effects • anaphylaxis • Post transfusion HA 20 % • Fever and chills 1 - 3% • Hepatitis C

  26. IV IgG • Benefits • Rapid response • effective in 80 % • Drawbacks • Expensive • Given by vein with prolonged infusion • Side effects • Temporary response

  27. Steroids • Inhibits phagocytosis of antibody coated platelets in spleen and prolongs platelet survival • Improves capillary resistance • Inhibits platelet antibody formation

  28. Steroids • Dose 2 - 4 mg/kg day • Benefits: • Inexpensive • Oral medication as outpatient • can vary dose easily

  29. Steroids • Drawbacks: • Slow response at low doses • side effects with prolonged use • Form of treatment for ALL

  30. Anti-RhD • Binds to Rh+ RBC and works in similar fashion to IV IgG • Benefits: • Rapid infusion (5 min) • Drawbacks: • Side effects • Drop in Hb • Pt needs to be Rh +

  31. Treatment of life-threatening bleeding • Platelet Transfusion • Steroids • IV IgG • Emergency splenectomy

  32. Outcome • 80 - 90% will have resolution within 6 months (acute ITP) • < 1% have CNS bleeding • Patients who have acute ITP resolve, rarely have recurrence • Patients “cured” by splenectomy can still make antibody. Concern for pregnancies

  33. Acute Vs Chronic ITP

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