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Primary immune thrombocytopenia (ITP) is an acquired, autoimmune haematological disease without an identifiable cause. The immune process is thought to be initiated by the generation of an autoantibody to platelet glycoproteins and sustained by an immune dysregulation of T-cells and cytokines, leading to progressive thrombocytopenia and increased risk of bleeding.1 Although most patients with ITP have mild to moderate thrombocytopenia and do not need treatment, adults with severe thrombocytopenia tend to suffer from recurrent relapses requiring medical intervention.
In 2009, a series of recommendations on terminology and outcome assessment in ITP were published by an international working group.2 The rationale was to establish an international nomenclature, to harmonise and to facilitate information exchange on clinical trial and management of ITP. Besides defining the thrombocytopenia of ITP as a platelet count <100×109/L and re-establishing the response criteria, the stage of ITP development have also been revised. Instead of the traditional, rather arbitrary, definition of acute (a self-limited form resolving within 6 months of diagnosis) and chronic (persistent disease lasting more than 6 months) phases, ITP has been recategorised into newly diagnosed (up to 3 months from diagnosis), persistent (3–12 months from diagnosis) and chronic (beyond 12 months from diagnosis), based on the likelihood of spontaneous remission and sustained response post treatment. None of these new phases, however, have been validated. In 2010, an International Consensus Report (ICR) on the management of ITP was published, followed by an updated evidence-based practice guideline on ITP from the American Society of Hematology (ASH) in 2011.3 4 The ICR and ASH management guidelines on ITP have offered the latest information based on the summary of clinical evidence and consensus from specialists in …
Contributors TN, AG, MS were involved in preparing and reviewing this editorial.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.
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