Abstract

Immune thrombocytopenic purpura is a comman disorder affecting children and adults. It is characterized by a low platelet count and mucocutaneous bleeding. ITP is classified as primary or secondary according to the presence of an underlying disorder or as acute or chronic according to duration of the disease. Although the accelerated platelet destruction in ITP occurs via antibody-mediated clearance, the clinical diagnosis of ITP is largely a diagnosis of exclusion. While humoral abnormalities in ITP are well defined, it is increasingly appearant that T cells play a major role in the onset of ITP. Genetic and enviromental factors and immune systems of patients that affect the etiology and the clinical progress of ITP have not been understood. Acute and chronic forms of the disease differ in that acute ITP is often preceded by an infectious illness and generally resolves spontaneously within a few weeks of initial presentation, whereas chronic form of the disorder defined as persistence of thrombocytopenia for greater than 6 months. Treatment modalities reported to date have been palliative rather than curative and corticosteroids and IVIG are used as the first choice drugs. Therapy of refractory patients remains controversial. A number of new agents are entering clinical trial in children and adults with ITP. The following review will focus on the pathophysiology, diagnostic approach and management of ITP.