Onkológia 6/2022

Immune thrombocytopenia in adults: modern approaches of treatment

Immune thrombocytopenia (ITP) is an autoimmune disease characterized by elevated immune-mediated platelet destruction in blood and reduced platelet production due to inhibition of megakaryocyte function. It is a disorder of accelerated antibody-mediated platelet destruction but also of impaired platelet production that results in thrombocytopenia. The diagnosis of ITP is based on the exclusion of other causes of isolated thombocytopenia. Secondary ITP can by present in patients with systemic autoimmune disorders, infections, solid tumours or lymphomas. Treatment should prevent severe bleeding episodes and should maintain a target platelet level > 20-30 x 109 /l at least for symptomatic patients. Treatment should be with minimal toxicity. For first-line treatment, cortikosteroids are recommended but half of the responders will relapse on follow-up. Thrombopoietin receptor agonists (TPO-RAs) have become available for second-line treatment of ITP. Some patients may have sustained off-treatment response after discontinuation of TPO-RA therapy. Splenectomy is associated with long-term treatment-free remissions however postsplenectomy patients may have higher risk for tromboembolism and infections.

Keywords: bleeding, corticosteroids, immune thrombocytopenia, rituximab, splenectomy, thrombopoietin receptor agonists