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.
Humans ; Purpura, Thrombocytopenic, Idiopathic ; classification ; immunology ; therapy

Adult ; Humans ; Purpura, Thrombocytopenic, Idiopathic ; diagnosis ; therapy

The diversity of diagnostic criteria of idiopathic thrombocytopenic purpura (ITP) makes it difficult to compare clinical trial results and exchange clinical experiences. To address this issue, an ITP international working group convened a consensus conference in Italy in October 2007, and some new consensus concerning the terminology, definition, phases, grading of severity, prognosis, and treatment were achieved. The treatment of ITP has been dramatically improved along with the introduction of novel therapeutic agents. Rituximab, a monoclonal anti-CD20 antibody that is able to deplete autoantibody-producing B lymphocytes, has been widely applied because of its high efficacy and safety. Recent evidences suggest that decreased platelet production may also contribute to the development of ITP. Therefore, novel thrombopoiesis-stimulating agents such as thrombopoietin-receptor agonists Romiplostim and Eltrombopag have become new therapeutic options for ITP.
Humans ; Purpura, Thrombocytopenic, Idiopathic ; diagnosis ; therapy

Adult ; Consensus ; Humans ; Purpura, Thrombocytopenic, Idiopathic/therapy*

Child ; China ; Humans ; Purpura, Thrombocytopenic, Idiopathic/therapy* ; Thrombocytopenia/therapy*

Idiopathic thrombocytopenic purpura (ITP) is a common hematological disease. It bleeds with peripheral blood platelet reduction as the main clinical manifestation, and manifests a chronic history in adult people. 11% - 35% ITP patients develop into a refractory course, which may be related with gene polymorphisms. There is currently no consensus on how best to manage refractory/relapsed ITP. In part, this reflects the need for individualized treatment due to the patients' requirements and their responsiveness to therapies. The objective of this review is to provide a clinically useful guide to current management strategies. This article summarizes all the treatment for refractory ITP, and highlights new therapies, including the anti-CD20 antibody, thrombopoietic agents, TPO receptor agonist and HSCT. The pancytoprotector shows good effect in the treatment of refractory and relapsed ITP in China. In a word, to give different treatments individually is most important.
Adult ; Humans ; Precision Medicine ; methods ; Purpura, Thrombocytopenic, Idiopathic ; therapy ; Recurrence

Objective: To report the clinical manifestations and laboratory features of five patients with congenital thrombotic thrombocytopenic purpura (cTTP) and explore its standardized clinical diagnosis and treatment along with a review of literature. Methods: Clinical data of patients, such as age of onset, disease manifestation, personal history, family history, and misdiagnosed disease, were collected. Treatment outcomes, therapeutic effects of plasma infusion, and organ function evaluation were observed. The relationship among the clinical manifestations, treatment outcomes, and ADAMTS13 gene mutation of patients with cTTP was analyzed. Additionally, detection of ADAMTS13 activity and analysis of ADAMTS13 gene mutation were explored. Results: The age of onset of cTTP was either in childhood or adulthood except in one case, which was at the age of 1. The primary manifestations were obvious thrombocytopenia, anemia, and different degrees of nervous system involvement. Most of the patients were initially suspected of having immune thrombocytopenia. Acute cTTP was induced by pregnancy and infection in two and one case, respectively. ADAMTS13 gene mutation was detected in all cases, and there was an inherent relationship between the mutation site, clinical manifestations, and degree of organ injury. Therapeutic or prophylactic plasma transfusion was effective for treating cTTP. Conclusions: The clinical manifestations of cTTP vary among individuals, resulting in frequent misdiagnosis that delays treatment. ADAMTS13 activity detection in plasma and ADAMTS13 gene mutation analysis are important bases to diagnose cTTP. Prophylactic plasma transfusion is vital to prevent the onset of the disease.
Female ; Pregnancy ; Humans ; Adult ; Blood Component Transfusion ; Plasma ; Purpura, Thrombotic Thrombocytopenic/therapy* ; Mutation ; Purpura, Thrombocytopenic, Idiopathic ; ADAMTS13 Protein/therapeutic use*

Humans ; China ; Consensus ; Purpura, Thrombocytopenic, Idiopathic/therapy* ; Thrombocytopenia/therapy* ; Thromboembolism/therapy*

The study was aimed to explore the relationship between infection of Helicobacter Pylorus (H. Pylorus) and etiology of idiopathic thrombocytopenic purpura (ITP) and evaluate whether H. Pylorus eradication can increase platelet count in patients with ITP. The data-bases of cqvip, Wanfang, TsingHua TongFang, CNKI and PubMed were searched, inclusion and exclusion criteria and heterogeneity test were determined. The studies of H. Pylorus infection and ITP were investigated with fixed effect mode Meta-analysis. Relationship between H. Pylorus infection and etiology of ITP, H. Pylorus eradication and curative effect of ITP were comprehensively and quantitatively evaluated. OR > 1 indicated that factor of exposure was the risk factor of disease; OR < 1 suggested that factor of exposure was the protective factor of disease; OR = 1 revealed that there were no correlation between factor of exposure and diseases. 95%CI was the confidence interval of total OR. The results showed that a total of 211 cases and 210 controls from 5 studies was included to evaluate the exposure of H. Pylorus between ITP patients and controls, the pooled OR was 1.73 (95%CI = 1.12 - 2.67); a total of 458 cases and 305 controls from 13 studies was included to evaluate the relationship between H. Pylorus eradication and curative effect of ITP, the pooled OR was 6.53 (95%CI = 4.44 - 9.61). It is concluded that H. Pylorus infection plays a role in the etiology of ITP. Eradication of H. Pylorus increases platelet count in patients with ITP. H. Pylorus eradication can be used as the first line measure to treat H. Pylorus-positive ITP.
Helicobacter Infections ; therapy ; Helicobacter pylori ; Humans ; Platelet Count ; Purpura, Thrombocytopenic, Idiopathic ; etiology ; microbiology ; therapy ; Treatment Outcome

Glucocorticoids ; pharmacology ; Humans ; Medicine, Chinese Traditional ; methods ; Purpura, Thrombocytopenic, Idiopathic ; therapy