Management of immune thrombocytopenic purpura in adults

R Stasi, D Provan - Mayo Clinic Proceedings, 2004 - Elsevier
R Stasi, D Provan
Mayo Clinic Proceedings, 2004Elsevier
Primary immune thrombocytopenic purpura (ITP), also referred to as idiopathic
thrombocytopenic purpura, is an organ-specific autoimmune disorder in which
antibodycoated 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 …
Primary immune thrombocytopenic purpura (ITP), also referred to as idiopathic thrombocytopenic purpura, is an organ-specific autoimmune disorder in which antibodycoated 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 × 109/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.
Elsevier