Treatment

• Prednisolone 2mg/kg PO in divided doses, slowly 5 over weeks; 70% respond well. Titrate to lowest dose to maintain Hb >7g/dL. Many achieve this on almost homeopathic doses despite true dependence. Around 10% need high dose maintenance and have trouble with side effects. 30% steroid resistant (try high dose methylprednisolone).

• Transfusion dependency usual in those who cannot be maintained on very low dose steroids. Need chelation to prevent iron overload. Use CMV -ve leucocyte-depleted packed RBC.

• Splenectomy not helpful (unless hypersplenism).

• Bone marrow transplantation worth considering for transfusion dependents with suitable donor; risk stratification as for severe thalassaemia (iron overload). Complicated decision due to chance of spontaneous remission even after years of transfusion dependency.

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