1 / 33

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:

jael-elliott
Télécharger la présentation

Thrombocytopenia

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  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

More Related