Primary
immune thrombocytopenia (
ITP) is an acquired blood disorder that causes a reduction in circulating platelets with the potential for
bleeding. The incidence of
ITP is slightly higher in adults and affects more women than men until 60 years, when males are more affected. Despite advances in basic science, primary
ITP remains a diagnosis of exclusion. The disease is heterogeneous in its clinical behavior and response to treatment. This reflects the complex underlying pathophysiology, which remains ill-understood. Platelet destruction plays a role in
thrombocytopenia, but underproduction is also a major contributing factor. Active
ITP is a proinflammatory
autoimmune disease involving abnormalities within the T and B regulatory cell compartments, along with several other immunological abnormalities. Over the last several years, there has been a shift from using immunosuppressive therapies for
ITP towards approved treatments, such as
thrombopoietin receptor agonists. The recent
COVID-19 pandemic has hastened this management shift, with
thrombopoietin receptor agonists becoming the predominant second-line treatment. A greater understanding of the underlying mechanisms has led to the development of several targeted
therapies, some of which have been approved, with others still undergoing clinical development. Here we outline our view of the disease, including our opinion about the major diagnostic and therapeutic challenges. We also discuss our management of adult
ITP and our placement of the various available
therapies.