Acute ITP in children usually resolves spontaneously and treatment is commenced only in life-threatening bleeding.
Adult acute ITP rarely resolves without treatment and 5-10% of patients develop a chronic ITP. The efficacy of steroids or intravenous immunoglobulin in reducing the duration of thrombocytopenia and the percentage of patients progressing to chronic ITP is unclear.
Chronic ITP rarely resolves spontaneously. First line treatment is with prednisolone:
- it is mandatory to perform a bone marrow aspiration before commencing steroids, to rule out leukaemia
- 20% of patients have a complete response
- 30% have a partial response and run a moderate thrombocytopenia of 30-100 requiring no further treatment or small doses of steroids
- the remaining 50% of patients, of which 30% would have initially shown a partial response to steroids, require splenectomy
- first line of therapy for ITP includes corticosteroids, sometimes in conjunction with IVIg or anti-Rh(D). While these are effective therapies, none reliably induce durable remission
- second line therapy for ITP may include Rituximab, splenectomy or thrombopoietin receptor agonists (TRAs)
- fostamatinib is a treatment option for chronic ITP
- is the first spleen tyrosine kinase (Syk) inhibitor approved for the treatment of chronic immune thrombocytopenia (ITP) in adult patients who have had an insufficient response to previous treatment
- by inhibiting Syk activation in macrophages, fostamatinib blocks autoantibody-mediated platelet phagocytosis
- splenectomy provides the greatest chance for long term remission, but its use is declining
Platelet transfusions are largely ineffective as exogenous platelets survive no better than endogenous ones. Transfusion should be given only in life-threatening haemorrhage to enhance haemostasis.
Reference:
- Kistangari G, McCrae KR. Immune thrombocytopenia. Hematol Oncol Clin North Am. 2013 Jun;27(3):495-520.
- Paik J. Fostamatinib: A Review in Chronic Immune Thrombocytopenia. Drugs. 2021 Jun;81(8):935-943