Consensus guidelines for the management of adult immune thrombocytopenia in Australia and New Zealand

Abstract

Introduction The absence of high quality evidence for basic clinical dilemmas in immune thrombocytopenic purpura (ITP) underlines the need for contemporary guidelines relevant to the local treatment context. ITP is diagnosed by exclusions, with a hallmark laboratory finding of isolated thrombocytopenia. Main recommendations Bleeding, family and medication histories and a review of historical investigations are required to gauge the bleeding risk and possible hereditary syndromes. Beyond the platelet count, the decision to treat is affected by individual bleeding risk, disease stage, side effects of treatment, concomitant medications, and patient preference. Treatment is aimed at achieving a platelet count > 20 × 109/L, and avoidance of severe bleeding. Steroids are the standard first line treatment, with either 6‐week courses of tapering prednisone or repeated courses of high dose dexamethasone providing equivalent efficacy. Intravenous immunoglobulin can be used periprocedurally or as first line therapy in combination with steroids. Changes in management as a result of this statement There is no consensus on choice of second line treatments. Options with the most robust evidence include splenectomy, rituximab and thrombopoietin receptor agonists. 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, we recommend platelet transfusions to achieve haemostasis, along with intravenous immunoglobulin and high dose steroids.

Treatment of immune thrombocytopenia in Australian adults: A multicenter retrospective observational study

“…In Australia, thrombopoietin agonists are eligible for PBS funding only for the treatment of chronic ITP following splenectomy. 13 While rituximab was not reimbursed by the PBS as a treatment for ITP during the study period, decreases in the cost of the drug during the study period could be expected to improve access for Australian patients. 13 Current international guidelines including the 2019 update to the ASH guidelines, as well as a recently published Australasian consensus guideline recommend the use of rituximab and TPO-As as splenectomy-sparing agents following the failure of first-line treatment.…”

“…Across all centers, initial treatment was in keeping with contemporaneous guideline‐recommended therapy 2 , 8 , 13 with most patients receiving either prednisone alone or in combination with IVIG. There was notable variability in the duration of prednisone weans across centers, with average duration ranging from 53 to 432 days; however, the details of prednisone weaning dosage were not captured in this study.…”

“… 13 While rituximab was not reimbursed by the PBS as a treatment for ITP during the study period, decreases in the cost of the drug during the study period could be expected to improve access for Australian patients. 13 …”

“…13 While rituximab was not reimbursed by the PBS as a treatment for ITP during the study period, decreases in the cost of the drug during the study period could be expected to improve access for Australian patients. 13 Current international guidelines including the 2019 update to the ASH guidelines, as well as a recently published Australasian consensus guideline recommend the use of rituximab and TPO-As as splenectomy-sparing agents following the failure of first-line treatment. 2,6,13 In comparison to previous studies investigating the effectiveness and tolerability of these agents, our data show similar rates of response/complete response for these agents [15][16][17] with a lower rate of side effects from rituximab than has previously been reported elsewhere.…”

İmmün trombositopenili erişkin hastalarda birinci basamak tedavide yüksek doz metilprednizolonun etkinliği

“…Our study's results indicate that HDP's relative effectiveness, low cost, and convenience of use continue to recommend it as a first-line therapy for adult patients with ITP. At the same time, too few studies to date have evaluated the effectiveness, HRQoL, and cost-effectiveness of the emergency treatment of ITP among adults18 , and recommendations for such situations have not been renewed in the guidelines for treatment, meaning that decisions and actions in clinically treating ITP are usually based on expert opinion. In light of both trends, multicenter, randomized controlled, prospective studies on best practices for treating ITP in adult patients are needed.…”