Primary immune thrombocytopenia (ITP) is an autoimmune disease characterized by increased destruction and impaired production of platelets caused by autoantibodies directed against the platelets and megakaryocytes and an increased risk of bleeding.
The incidence in adults has been estimated between 1.6 and 3.95 x 100 000 subjects per year, depending on the selected diagnostic cut-off, ranging from <100 to <50 x 10^9/L. The incidence tends to increase with age, and females have shown a higher incidence than males in patients younger than 60 years. In Spain, epidemiologic data regarding ITP are scarce. The estimated incidence in adults is 1.6 to 6.6 new cases of chronic ITP x 100 000 subjects per year.
Signs and symptoms of ITP vary widely. While many patients have either no symptoms or minimal bruising, others experience serious bleeding, including gastrointestinal hemorrhage, extensive skin, and mucosal hemorrhage, or intracranial hemorrhage. The severity of hemorrhage correlates to some extent but not completely with bleeding risk.
The treatment goal in chronic ITP is to increase and maintain platelet count within a safe range to prevent bleeding and also improving health-related quality of life. Current guidelines recommend corticosteroids as first-line therapy as these agents have been shown to afford the most rapid rise in the platelet count. Splenectomy is one of several second-line therapies, mainly based on immunosuppressives.
Recent consensus and guidelines recommend the thrombopoietin receptor agonists (TPO-RAs), romiplostim and eltrombopag, as second-line or further anti-ITP treatment.
The TPO-RAs are currently used to treat relapsed patients with ITP who are at risk of bleeding and who have already failed at least one ITP therapy. These agents are sometimes used off-label before an invasive procedure and during the persistent phase of severe ITP in patients without response to corticosteroids and/or intravenous immunoglobulin.
Nevertheless, there are many new drugs with potential use in ITP that probably will compete with TPO-RAs in a near future.
Our aim with this project is to call for old and new ITP drug reports and for new insights regarding the pathophysiology of ITP. The scope of this Research Topic is to collect manuscripts on old (classic) and new therapies with a special focus on ITP diagnosis.
We consider combo therapies in ITP need more trials and, if not, more daily practice clinical data to support its use, especially in refractory/relapsing ITP.
A similar situation appears when trying to know updated and feasible information about new and latest drug use out of the clinical trial setting.
On the other hand, the biological basis of ITP still remains unclear to date. For this reason, we would like to publish new points of interest of ITP that may have been overlooked before.
Topic Editor T.J.G.-L. has received research grants from Amgen, Momenta and Novartis. Topic Editor D.P. has received research support and honoraria from Amgen and Novartis. The other Topic Editors declare no competing interests with regard to the Research Topic subject
Primary immune thrombocytopenia (ITP) is an autoimmune disease characterized by increased destruction and impaired production of platelets caused by autoantibodies directed against the platelets and megakaryocytes and an increased risk of bleeding.
The incidence in adults has been estimated between 1.6 and 3.95 x 100 000 subjects per year, depending on the selected diagnostic cut-off, ranging from <100 to <50 x 10^9/L. The incidence tends to increase with age, and females have shown a higher incidence than males in patients younger than 60 years. In Spain, epidemiologic data regarding ITP are scarce. The estimated incidence in adults is 1.6 to 6.6 new cases of chronic ITP x 100 000 subjects per year.
Signs and symptoms of ITP vary widely. While many patients have either no symptoms or minimal bruising, others experience serious bleeding, including gastrointestinal hemorrhage, extensive skin, and mucosal hemorrhage, or intracranial hemorrhage. The severity of hemorrhage correlates to some extent but not completely with bleeding risk.
The treatment goal in chronic ITP is to increase and maintain platelet count within a safe range to prevent bleeding and also improving health-related quality of life. Current guidelines recommend corticosteroids as first-line therapy as these agents have been shown to afford the most rapid rise in the platelet count. Splenectomy is one of several second-line therapies, mainly based on immunosuppressives.
Recent consensus and guidelines recommend the thrombopoietin receptor agonists (TPO-RAs), romiplostim and eltrombopag, as second-line or further anti-ITP treatment.
The TPO-RAs are currently used to treat relapsed patients with ITP who are at risk of bleeding and who have already failed at least one ITP therapy. These agents are sometimes used off-label before an invasive procedure and during the persistent phase of severe ITP in patients without response to corticosteroids and/or intravenous immunoglobulin.
Nevertheless, there are many new drugs with potential use in ITP that probably will compete with TPO-RAs in a near future.
Our aim with this project is to call for old and new ITP drug reports and for new insights regarding the pathophysiology of ITP. The scope of this Research Topic is to collect manuscripts on old (classic) and new therapies with a special focus on ITP diagnosis.
We consider combo therapies in ITP need more trials and, if not, more daily practice clinical data to support its use, especially in refractory/relapsing ITP.
A similar situation appears when trying to know updated and feasible information about new and latest drug use out of the clinical trial setting.
On the other hand, the biological basis of ITP still remains unclear to date. For this reason, we would like to publish new points of interest of ITP that may have been overlooked before.
Topic Editor T.J.G.-L. has received research grants from Amgen, Momenta and Novartis. Topic Editor D.P. has received research support and honoraria from Amgen and Novartis. The other Topic Editors declare no competing interests with regard to the Research Topic subject