Primary immune thrombocytopenia (ITP) is an organ-specific autoimmune disorder characterized by autoantibody-mediated enhanced platelet destruction and dysmegakaryocytopoiesis. B cells have been demonstrated to play critical roles in the pathophysiology of ITP. B-cell-activating factor (BAFF) and a proliferation-inducing ligand (APRIL) are crucial cytokines supporting survival and differentiation of B cells, and dysregulation of BAFF/APRIL is involved in the pathogenesis of B-cell related autoimmune diseases including ITP. Currently ongoing clinical trials using BAFF and/or APRIL-blocking agents have yielded positive results in human systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA), further confirming the pathological role of BAFF/APRIL in autoimmunity. This review will describe the function of BAFF/APRIL and address the feasibility of BAFF/APRIL inhibition in the management of ITP.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1053/j.seminhematol.2013.03.021 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Autoantibodies against phosphatidylserine and DNA during canine Dirofilaria immitis infection.
Vet Parasitol
January 2025
Biological Sciences Department, New York City College of Technology, City University of New York, Brooklyn, NY, United States; Biology PhD Program, CUNY Graduate Center, New York, NY, United States. Electronic address:
Heartworm infection caused by Dirofilaria immitis induces a devastating disease that greatly affects the global canine population. The mechanism leading to heartworm pathology has been attributed to be mostly by mechanical damage of the worm to the dog´s vascular system and immune-mediated, but the latter processes are not completely understood. Autoantibodies targeting host molecules such as lipids and nucleic acids have been described with pathological roles during malaria and COVID-19 and mediating anemia and thrombocytopenia.
View Article and Find Full Text PDF[High mobility group protein B1(HMGB1) promotes myeloid dendritic cell maturation and increases Th17 cell/Treg cell ratio in patients with immune primary thrombocytopenia].
Xi Bao Yu Fen Zi Mian Yi Xue Za Zhi
January 2025
Hematologic Disease Center, First Affiliated Hospital of Xinjiang Medical University, Xinjiang Uygur Autonomous Region Research Institute of Hematology, Xinjiang Medical University, Urumqi, Xinjiang Uygur Autonomous Region, Wulumuqi 830011, China. *Corresponding author, E-mail:
Objective This study investigated the regulatory effect of high mobility group protein B1 (HMGB1) in the peripheral blood of patients with primary immune thrombocytopenia (ITP) on myeloid dendritic cells (mDC) and Th17/regulatory T cells (Treg) balance. Methods The study enrolled 30 newly diagnosed ITP patients and 30 healthy controls.Flow cytometry was used to measure the proportion of mDC, Th17, and Treg cells in the peripheral blood of ITP patients and healthy controls.
View Article and Find Full Text PDFPredictors of Splenectomy Response in Immune Thrombocytopenia: A Multicentric Italian Study.
J Clin Med
December 2024
Hematology and Clinical Immunology Unit, Department of Medicine, University of Padua, 35122 Padua, Italy.
: Splenectomy leads to a high rate of remission in chronic primary immune thrombocytopenia (ITP), but its unpredictable long-term positive outcomes and that it is a irreversible surgical approach discourage clinicians and patients. The identification of predictors of response may redefine the timing of splenectomy. In this retrospective, multicentric study we aimed to investigate clinical-histological predictors of splenectomy response in ITP patients and provide an easy-to-use score to predict splenectomy response in ITP.
View Article and Find Full Text PDFEltrombopag for the treatment of refractory connective tissue disease-related thrombocytopenia: a pilot study of 52 cases.
Arthritis Res Ther
January 2025
Department of Rheumatology and Immunology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Background: The objective of this study was to investigate the therapeutic effectiveness and safety profile of Eltrombopag, a thrombopoietin receptor agonist, as prolonged therapy in refractory CTD-ITP patients.
Methods: We conducted a pilot observational study of Eltrombopag in CTD-ITP patients who were unresponsive to or intolerant of conventional medications. Eltrombopag was administered orally at 25-75 mg/qd and adjusted on the basis of tolerance and efficacy until a minimum dosage of 25 mg/qd was reached.
Exosome-Mediated Lectin Pathway and Resistin-MIF-AA Metabolism Axis Drive Immune Dysfunction in Immune Thrombocytopenia.
Adv Sci (Weinh)
January 2025
Translational Medical Center, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, 450001, China.
Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by reduced platelet levels and heightened susceptibility to bleeding resulting from augmented autologous platelet destruction and diminished thrombopoiesis. Although antibody-mediated autoimmune reactions are widely recognized as primary factors, the precise etiological agents that trigger ITP remain unidentified. The pathogenesis of ITP remains unclear owing to the absence of comprehensive high-throughput data, except for the belated emergence of autoreactive antibodies.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!