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IDIOPATHIC THROMBOCYTOPENIA PURPURA (ITP) in children : summarized


IDIOPATHIC THROMBOCYTOPENIA PURPURA (ITP)

Dr Zubair Bhutta

Ø  Most common cause of acute onset of thrombocytopenia in otherwise well child
Epidemiology:
Ø  more often in late winter and spring (peak season of viral resp. illness)
Ø  Average age is 1- 4 years
Ø  Mostly after 1-4 weeks of viral infection – auto antibodies against platelets surface
Ø  Most common viruses, Epstein-Barr viruses(short duration) and HIV (Chronic)
Ø  Also association with H.pylori infection in children
Sign and Symptoms:  (Classical Presentation of ITP)
Ø  1-4 yr healthy child has sudden onset of generalized petechiae and purpura
Ø  Bleeding from gums,epistaxis and mucosal membranes.
Ø  Platelet count (<10X109/L)
Ø  Hx of preceding viral infection 1 – 4 weeks back
Ø  No physical findings on examination other than patechiae and purpura.
Investigations:
Ø  CBC – Dec.Platelets but normal Hb and WBC
Ø  Periphral Blood Smear – normal or large platelets (megakaryocytes)
Ø  Bone marrow aspiration / biopsy –immature megakaryocytes
(Indication for BMA : 1)abnormal WBC 2)Hx and Examination suggestive of malignancy 3)HIV studies in high sexually active teens 4)Unexplained anemia 5)Plt. Antibodies testing 6)Direct Coomb’s test – if unexplained anemia with thrombocytopenia

Classification:   severity of bleeding on the basis of sign and symptoms but NOT platelet count

Minimal
Mild
Moderate
Severe
Bruising and petechiae
No
Minor
More Sever – menorrhagia occasionally
Bleeding episodes, menorrhagia and malena
Epistaxis
No
Occasional/minor
More troublesome
Severe
Quality of life
No
Little Interferance
Affected life
Hospitalization


OUTCOMES:
Ø  70 – 80 Percent spontaneously resolved within 6 months
Ø  Fewer than 1 percent develop intracranial hemorrhage
Ø  20 Percent Acute ITP become Chronic ITP

D/Ds: 1)Medication exposure 2)Early aplastic exposure i.e fanconi anemia 3)HUS 4)DIC 5)Heparin induced thrombocytopenia 6)SLE 7)HIV infection 8)Common variable of immunodeficiency
Management and Treatment:   No established benefit of treatment and PLT Tx is contraindicated unless life threatening bleeding occurs
1)      Education and counseling of family and patient
2)      Single dose if IVIG  Or IVIG for 2 DAYS:
Dose: 0.8 – 1.0 g/Kg     Side effects: Headache,Vomiting and IVIG induced aseptic meningitis
3)      Corticosteroids: Prednisone – Short course until Plt rise to >20X109/L
Dose:1 – 4 mg/kg/day  Short term Side Effects: Hypertension Long term side effects:Growth failure, Diabetes Mellitus,Osteoprosis
4)Intravenous Anti-D Therapy: Only  for Rh positive patients
Dose:50 – 75 ug/Kg     Side effects: Mild haemolytic anemia

5)Splenectomy: only indicated in 1 of these circumstances *older child >4 yrs with severe ITP lasted >1 yr *Symptoms not controlled with medical therapy * Life threatening Intracranial hemorrhage

6)Rituximab: Alternative to splenectomy

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