Management of Immune Thrombocytopenic Purpura in Adults

Abstract

Primary immune thrombocytopenic purpura (ITP), also referred to as idiopathic thrombocytopenic purpura, is an organ-specific autoimmune disorder in which antibody-coated or immune complex-coated platelets are destroyed prematurely by the reticuloendothelial system, resulting in peripheral blood thrombocytopenia. The disease is heterogeneous with regard to its severity and clinical course and is unpredictable in its response to therapy. Although the basic underlying pathophysiology of ITP has been known for more than 50 years, current treatment guidelines are based on expert opinion rather than on evidence because of a lack of high-quality clinical trials and research. The only patients for whom treatment is clearly required are those with severe bleeding and/or extremely low platelet counts (< 10 x 10(9)/L). Treatment of patients with ITP refractory to corticosteroids and splenectomy requires careful evaluation of disease severity, patient characteristics related to risk of bleeding, and adverse effects associated with treatment. Clinical trials with numerous new agents are under way, which we hope will add more effective and targeted strategies to our therapeutic armamentarium. We describe a logical and structured approach to the clinical management of ITP in adults, based on a literature review and our personal experience.

Romiplostim safety and efficacy for immune thrombocytopenia in clinical practice: 2-year results of 72 adults in a romiplostim compassionate-use program

“…[1][2][3][4][5] ITP signs and symptoms vary widely. Some patients have no symptoms or minimal bruising, whereas others experience severe bleeding.…”

A prospective observational study of dengue fever with thrombocytopenia with reference to treatment

“…The possible pathogenesis of thrombocytopenia in dengue fever are enhanced peripheral destruction of antibody coated platelets, acute bone marrow suppression leading to a megakaryocytic condition and increased destruction of platelets by the reticuloendothelial system which helps to plan for the future research in severe complications of dengue fever. 2,3 Our study demonstrated that the all three groups are similar in the increasing platelet count in dengue fever with thrombocytopenia patients (Figure 2). The rise in mean platelet count remained similar in all the groups.…”

“…Other mechanisms are acute bone marrow suppression leading to a megakaryocytic condition and increased destruction of platelets by the reticuloendothelial system. 2,3 Dengue fever causes thrombocytopenia commonly which often leads to life threatening complications like dengue hemorrhagic fever (DHF) and dengue shock syndrome (DSS). Both are fatal complications leading to hemorrhagic diathesis and circulatory collapse.…”

Eltrombopag: an update on the novel, non-peptide thrombopoietin receptor agonist for the treatment of immune thrombocytopenia

“…It is characterised by a transient or persistent decrease in platelets and an increase in the risk of bleeding [1][2][3][4][5]. The estimated incidence of ITP is 100 cases per 1 million persons per year, with approximately 50% of these cases occurring in children [2,6].…”

“…In adults, the incidence is higher in women than in men [2], whereas the reverse is true in children, where ITP predominates in young male [7]. Clinical presentation varies from asymptomatic or mild symptoms (bruising and petechiae) to mucocutaneous bleeding that can become severe [5,8].…”