Dr Phillip Choi
Australian guidelines have been developed to assist clinicians treating adults with immune thrombocytopenic purpura (ITP)
Consensus recommendations have been developed by local clinicians based on the GRADE approach to evaluating evidence, endorsed by the Thrombosis and Haemostasis Society of Australia and New Zealand (THANZ) and ITP Australia.
Led by Dr Phillip Choi, a Consultant Haematologist at The Canberra Hospital and Clinical Director of the National Platelet Research and Referral Centre, the guideline developers say most of the recommendations are based on low level evidence, but this only highlights the need for practical advice on how to address the basic clinical dilemmas in ITP with a “crowded but flawed treatment armamentarium”.
The guidelines, published in the MJA, cover issues such as initial investigations and decisions on when to start treatment, first and second line treatments, supportive acre and special situations such as treatment of emergency bleeding in ITP and interventions to raise platelet counts before surgery or procedures.
Recommendations are also provided on pregnancy and ITP, and on management of vascular disease and VTE and severe thrombocytopenia in people with ITP.
Detailed recommendations are provided on the role of treatments such as splenectomy and agents such as rituximab, TPO‐Ras and other therapies such as mycophenolate mofetil and dapsone.
According to the guidelines, the overall aim of treatment is to achieve a platelet count > 20 × 109/L, and avoidance of severe bleeding.
Steroids are recommended as the standard first line treatment, and intravenous immunoglobulin may be used periprocedurally or as first line therapy in combination with steroids.
The authors say the main changes in management as a result of their recommendations relate to second line treatments, where there is currently no consensus on best practice.
“Options with the most robust evidence include splenectomy, rituximab and thrombopoietin receptor agonists,” they note.
“Other therapies include azathioprine, mycophenolate mofetil, dapsone and vinca alkaloids. Given that up to one‐third of patients achieve a satisfactory haemostatic response, splenectomy should be delayed for at least 12 months if possible.”
In life‐threatening bleeding, they recommend platelet transfusions to achieve haemostasis, along with intravenous immunoglobulin and high dose steroids.
However the guideline authors acknowledge the recommendations are likely to require updating in the near future as novel therapies become available for ITP, including those targeting Fc neonatal receptors, Bruton tyrosine kinase (BTK) and spleen tyrosine kinase (SYK) signalling, and complement inhibition.
“Anticipating the best way to incorporate these new modalities into our already crowded but flawed treatment armamentarium remains a challenge for us in the future,” they conclude.