Epidemiology, pathophysiology, and initial management of chronic immune thrombocytopenic purpura.

PURPOSE: The incidence and epidemiology, the pathogenesis, the clinical symptoms and diagnosis and the first-line therapies for the management of chronic immune thrombocytopenic purpura (ITP) are discussed. In addition, the recommendations of two expert panels for the management of ITP are summarized.

SUMMARY: The diagnosis and management of chronic ITP are a challenge to the clinician caring for patients with this disease. Because the pathophysiology of ITP is not completely understood, a variety of medical interventions have been utilized in the management of ITP. National guidelines have established that oral corticosteroids are considered to be first-line therapy for chronic ITP. In addition, the use of intravenous immune globulin has demonstrated efficacy in the treatment of the disease. Intravenous methylprednisolone, anti-D immunoglobulin, and splenectomy have been utilized in recurrent or refractory cases. The use of other immunosuppressant medications and newer thrombopoietin stimulating agents may offer additional treatment options, as presented in the subsequent article.

CONCLUSION: The initial management of chronic ITP should consist of the use of oral corticosteroids according to national guidelines. In the absence of a response to this first-line therapy, intravenous gamma globulin, intravenous methylprednisolone, anti-D immunoglobulin, or splenectomy may be considered. These treatments may also be utilized to manage recurrent cases of ITP, prior to consideration of second-line therapies.