Thrombocytopenia
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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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Thrombocytopenia

Thrombocytopenia

ITP


Platelets

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


Platelets1

Platelets

  • The types of bleeding seen in platelet disorders include:

    • Skin and mucous membranes

      • Petechiae and purpura

    • Epistaxis, hematuria, menorrhagia

    • GI bleeding

    • Rarely ICH


Platelets2

Platelets

  • Normal number: 150,000 - 400,000

  • Distribution: 1/3 in spleen, 2/3 in PBL

  • Life Span: 7 - 10 days


Causes of thrombocytopenia

Causes of Thrombocytopenia

  • Decreased production

  • Increased destruction


Decreased production

Decreased Production

  • Congenital

    • TAR syndrome

    • Fanconi’s anemia

    • Amegakaryocytic thrombocytopenia


Decreased production1

Decreased Production

  • Acquired

    • Infiltrative process

      • ALL, NBL etc

    • Aplastic anemia

    • Nutritional deficiencies

    • Drugs


Increased platelet destruction

Increased Platelet Destruction

  • Immune

    • Neonatal alloimmune Thrombocytopenia

    • Drug related

    • HIV

    • Post-transfusion purpura

    • autoimmue

      • SLE

      • ITP


Non immune

Non-immune

  • Hemolytic-Uremic Syndrome

  • DIC

  • Cyanotic Heart Disease


Qualitative platelet disorder

Qualitative Platelet Disorder

  • Wiskott Aldrich Syndrome

  • Bernard - Soulier Syndrome

  • May - Hegglin Anomaly


Sequestration

Sequestration

  • Kasabach - Merrit Syndrome

  • Hypersplenism


Thrombocytopenia

ITP


Thrombocytopenia

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


Clinical presentation

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


Differential diagnosis

Differential Diagnosis

  • Drug induced thrombocytopenia

  • Viral related

  • HUS

  • Wiskott-Aldrich Syndrome

  • Infiltrative disorders


History

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


History1

History

  • Bone pain

  • Headache or Neurological changes

  • joint Sx

  • Previous Hx of low platelets or family history of low platelets


Physical exam

Physical Exam

  • Location and types of bleeding

  • Fundiscopic and Neuro exam

  • Lymphadenopathy and/or splenomegly

  • Joint and skin exam


Laboratory data

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


Outcome

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


Treatment

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


Treatment options

Treatment Options

  • IV IgG

  • Steroids

  • IV anti RhD (Winrho)

  • Splenectomy

  • Other


Iv igg

IV IgG

  • IV IgG


Iv igg1

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


Iv igg2

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


Iv igg3

IV IgG

  • Benefits

    • Rapid response

    • effective in 80 %

  • Drawbacks

    • Expensive

    • Given by vein with prolonged infusion

    • Side effects

    • Temporary response


Steroids

Steroids

  • Inhibits phagocytosis of antibody coated platelets in spleen and prolongs platelet survival

  • Improves capillary resistance

  • Inhibits platelet antibody formation


Steroids1

Steroids

  • Dose 2 - 4 mg/kg day

  • Benefits:

    • Inexpensive

    • Oral medication as outpatient

    • can vary dose easily


Steroids2

Steroids

  • Drawbacks:

    • Slow response at low doses

    • side effects with prolonged use

    • Form of treatment for ALL


Anti rhd

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 +


Treatment of life threatening bleeding

Treatment of life-threatening bleeding

  • Platelet Transfusion

  • Steroids

  • IV IgG

  • Emergency splenectomy


Outcome1

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


Acute vs chronic itp

Acute Vs Chronic ITP


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