Recent progress of study on mechanism of immune thrombocytopenic purpura and its clinical treatment - review / 中国实验血液学杂志

ABSTRACT

Immune thrombocytopenia purpura (ITP) is a disorder mediated by antiplatelet antibodies and characterized by accelerated destruction of platelets and impaired platelet production. The mainstay therapies for ITP have included corticosteroids, the immune globulin intravenous immunoglobulin and IV anti-D (monoclonal antibodies against the D antigen of the Rh system), vinblastine or a monoclonal anti-CD20 antibody that transiently depletes CD20(+) B cells, danazol, cyclophosphamide and even splenectomy to refractory one. Most of ITP patients responded to those treatment, while more than 30% of whom may relapse sooner or later. The recombinant forms of human TPO were discontinued from human use in clinic because recipients of these agents developed significant thrombocytopenia secondary to production of neutralizing antibodies that cross-reacted with endogenous TPO. All above mentioned treatments have side effects and severe infection may arise post splenectomy. The more powerful treatment with less side effects are needed. There are two TPO receptor agonist, AMG531 and Eltrompobag, have approved in Europe for the treatment of ITP. Both of them can improve the differentiation of megakaryocyte and platelets production. Combination treatment including pancytoprotector shows good effect in the treatment of refractory and relapsed ITP in China. Altogether, individual treatment of ITP is the contemporary trend in both clinical and preclinical practice. In this review the pathogenesis of ITP and its clinical therapies were reviewed, the individual regiments for treating ITP patients were discussed.