[Chronic idiopathic thrombocytopenic purpura. Current therapy concept and introduction to pathophysiologic, clinical and diagnostic aspects]

Abstract

Pathophysiology: Chronic idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by platelet destruction due to an antiplatelet autoantibody, usually of the IgG class, which coats autologue platelets and leads to their elimination by the reticuloendothelial system (RES). While in childhood ITP is more usually an acute and self-limiting problem which needs no drug treatment, adult ITP is a relatively common chronic hematological disease.

Treatment: Treatment aimes at inhibition of antibody-production and binding on thrombocytes and thrombocyte phagocytosis by the RES. Therapy should result in a platelet count of > 100,000/microliter or at least in stabilization of the platelet count without bleeding. Therapeutic approaches were divided into emergency and long-term treatment. In patients who require non-emergency treatment conventional-dose corticoids (1 to 2 mg/kg/d prednisone) are recommended as initial treatment, whereas pulsed high-dose dexamethason is recently reported to be effective in refractory ITP. After unsuccessful splenectomy or if treatment with gammaglobulins fails alternative and partly experimental therapies may have to be used.

Conclusion: Treatment of adult ITP includes established medical, immunological and surgical measurements. Their application depends on diseases progression as well as imminent or manifest complications. Remission is achieved in up to 75% of all patients. Alternative treatments remain for refractory cases.