3. Clinical
Presentation
A 2-year-old boy is brought to hospital because
of gum bleeding and hematuria for 2 days.
One week before admission he was seen in the
clinic for cough and fever
4. Examination
Afebrile
Pink Mucous Membranes
Petechiae evident on the face and abdomen.
Urine shows frank (gross) hematuria
5. Investigations
Hb 11.0 g/dl normal adult 13.5-17.5 2year old 10.5-13.5
WBC 9.5x10^9/L normal adult 4-11 2 year old 5-15.5
Platelets- 5x10^9/L normal adult 140-400 2 year old 214-483
7. ITP
Increased destruction of platelets
ITP is categorized as:
Chronic Idiopathic Thrombocytopenic Purpura and
AcuteThrombocytopenic Purpura
Acute is mostly seen in children, sometimes following
a vaccination or infection.
Most cases caused by nonspecific immune complex
attachments to platelets.
8. ITP
Spontaneous remissions are usual, but 5-10% of cases
can become chronic, lasting more than 6 months
There is a rare risk of cerebral hemorrhaging.
9. ITP
Pathogenesis
Platelet Autoantibodies such as IgG result in the
premature removal of platelets from the circulation by
macrophages of the reticuloendothelial system
Most times, the antibody is directed against the
glycoprotein GP IIb/IIIa or Ib complex.
10. ITP
Pathogenesis
The normal life span of a platelet is 10 days but in ITP it
is reduced to a few hours.
Total megakaryocyte mass and platelet turnover are
increased by 5 times normal
12. ITPClinical
Features and
Diagnosis
Petechial Hemorrhage
Easy Bruising
Mucosal bleeding
Epistaxis
Gum
Platelet Count 10-100 x 10^9/L
Normal Hb andWBC unless IDA from blood loss
Total megakaryocyte mass and platelet turnover are
increased by 5 times normal
15. Management
of ITP
If there was no hemorrhaging, no treatment would be
necessary.
This was why the child was sent home for observation after
the initial visit to the clinic for fever and cough.
Treatment is with steroids and/or intravenous
immunoglobulin to increase platelet count to
30x10^9/L, the threshold for bruising with low
platelets.
Avoid activities that would cause further trauma or
bruising
ITP is a disorder of platelet amount, where there is an increased destruction o f platelets
The pathogenesis of thrombocytopenia in autoimmune thrombocytopenic purpura. Platelets coated by antibodies are phagocytosed by macrophages. The actions of thrombopoietin (TPO) and thrombopoietin receptor agonists (TPO‐RA) (thrombo mimetics) are shown. These are orally active or given by injection and act to increase platelet production.
Iron deficiency anemia
(Since this is a diagnosis of exclusion, you would want to rule out differentials that could cause similar clinical presentation)?