Management

Newly presenting

Seldom require urgent therapy though this is frequently given, either polyvalent IVIg 0.8g/kg (single dose) or prednisolone 4mg/kg. Never justified in the absence of obvious bleeding since neither therapy without risk. Simple observation for spontaneous recovery should be preferred.

Chronic

No therapy is needed based on platelet count alone. Absence of symptoms and signs is sufficient. Excessive restriction of activities is seldom justified. Open access to expert help and advice provides reassurance to families and teachers. If therapy required to control symptoms (recurrent nosebleeds, menorrhagia) try local measures or hormonal control. Regular IVIg or steroids seldom effective and may produce more problems than untreated disease. For the most difficult cases (very rare) splenectomy still worth considering, though post-splenectomy mortality may be > than that of untreated ITP. Newer therapies include danazol and rituximab, though experience still anecdotal and long-term risks not yet evaluated.

Life-threatening haemorrhage or other emergency

Risk of life-threatening haemorrhage very small (<1/1000 in first week after diagnosis) though is a function of a platelet count <10-20 x 109/L and the time exposed to this. Risk consequently rises in rare children with chronic unremitting severe disease for >1-2 years for whom more adventurous therapy (splenectomy, rituximab) can be contemplated, though risk still small and those of treatment may be higher. If a large intracranial (ICH) or other catastrophic bleed occurs, this can be dealt with by simultaneous massive platelet transfusion, IVIg, IV methylprednisolone and (if the diagnosis is beyond doubt) emergency splenectomy. Mortality of major ICH less than 50% given active therapy.

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