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Autoimmune Thrombocytopenia

C E Neunert, J M Despotovic
Autoimmune cytopenias (AIC) post-hematopoietic stem cell transplant (HSCT) are rare but exceptionally challenging complication. We conducted a comprehensive literature review and identified a pooled incidence of post-HSCT autoimmune hemolytic anemia and/or immune thrombocytopenia of 2.66% (SE = 0.27) in pediatric patients. Nonmalignant disease, unrelated donor transplant, peripheral or cord blood stem cell source, conditioning regimen without total body irradiation, and presence of chronic graft-versus-host disease were prominent risk factors...
December 10, 2018: Pediatric Blood & Cancer
Vincent M Vacca
Acquired autoimmune thrombotic thrombocytopenic purpura (TTP)-the most common form of TTP-is a life-threatening hematologic disease characterized by hemolytic anemia and thrombocytopenia. Acquired autoimmune TTP can cause signs and symptoms of neurologic and other organ involvement, with mortality approaching 90% if the disease is not promptly recognized and treated. Since the introduction of plasma exchange in 1991, the acquired autoimmune TTP survival rate has increased to 78%.
December 10, 2018: Nursing
Giselle Castaneira, Kelley Rojas, Yehuda Galili, Zachary Field, Ariel Perez-Perez, Mario Madruga, Steve J Carlan
: Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder defined as low platelet count with normal bone marrow in the absence of other causes of thrombocytopenia. It is caused by autoantibodies binding to several platelet surface antigens which lead to premature destruction of the platelet by the reticuloendothelial system. ITP can be primary or secondary, and it is known to be associated with various infections and drugs. K2/Spice, or synthetic marijuana, acts on cannabinoid receptors CB1 (cannabinoid type-1) and CB2 (cannabinoid type-2) with increased binding capacity compared to marijuana...
November 29, 2018: Journal of Addiction Medicine
Bryan Smith, Louis Christodoulou, Alison Clargo, Alison Eddleston, Kevin Greenslade, Daniel Lightwood, Anthony Shock, Kerry Tyson, Frank R Brennan
Primary immune thrombocytopenia (ITP) is an autoimmune disease characterized by pathogenic immunoglobulin G (IgG) autoantibodies that bind to platelets, causing their phagocytic removal and leading to reductions in platelet number. The neonatal Fc receptor (FcRn) selectively salvages and recycles IgG, including pathogenic IgG, thereby extending the half-life of IgG in plasma. Two anti-mouse FcRn monoclonal antibodies (mAb) (4470 and 4464) were generated to evaluate the effect of inhibiting IgG recycling. Statistically significant reductions in plasma IgG concentration were observed upon administration of 4470 (10, 30 and 100 mg/kg) in wild-type mice...
December 4, 2018: International Immunopharmacology
Nicolas Delanoy, Jean-Marie Michot, Thibault Comont, Nora Kramkimel, Julien Lazarovici, Romain Dupont, Stéphane Champiat, Claude Chahine, Caroline Robert, Charles Herbaux, Benjamin Besse, Aude Guillemin, Christine Mateus, Patricia Pautier, Philippe Saïag, Emanuela Madonna, Marie Maerevoet, Jean-Christophe Bout, Charlotte Leduc, Pascal Biscay, Gilles Quere, Charlée Nardin, Mikael Ebbo, Laurence Albigès, Grégoire Marret, Virginie Levrat, Cécile Dujon, Jacques Vargaftig, Salim Laghouati, Laure Croisille, Anne-Laure Voisin, Bertrand Godeau, Christophe Massard, Vincent Ribrag, Aurélien Marabelle, Marc Michel, Olivier Lambotte
BACKGROUND: Anti-programmed cell death 1 (PD-1) and anti-programmed cell death ligand 1 (PD-L1) antibodies are novel immunotherapies for cancer that can induce immune-related adverse events (irAEs). These adverse events can involve all organs, including the haemopoietic system. Thus far, haematological irAEs (haem-irAEs) have not been extensively characterised. This study aims to provide a comprehensive report of the haem-irAEs induced by anti-PD-1 or anti-PD-L1. METHODS: In this descriptive observational study, we included consecutive patients aged at least 18 years with grade 2 or worse haem-irAEs induced by anti-PD-1 or anti-PD-L1 immunotherapy registered in three French pharmacovigilance databases: the Registre des Effets Indésirables Sévères des Anticorps Monoclonaux Immunomodulateurs en Cancérologie (REISAMIC; a prospective registry of patients treated with anti-PD-1 or anti-PD-L1 at a single centre), the ImmunoTOX committee of Gustave Roussy (a national referral database of suspected irAEs in patients treated with immunotherapy), and the registry of the Centre de Référence des Cytopénies Auto-Immunes de l'Adulte (CeReCAI; a national database of autoimmune cytopenias)...
December 4, 2018: Lancet Haematology
Dolores Pérez, Ljudmila Stojanovich, Laura Naranjo, Natasa Stanisavljevic, Gordana Bogdanovic, Manuel Serrano, Antonio Serrano
Background: Antiphospholipid syndrome (APS) is an acquired autoimmune disorder defined by the presence of both clinical (thromboembolic events or pregnancy morbidity) and laboratory (antiphospholipid antibodies, aPL) manifestations. Despite their importance, several clinical manifestations strongly associated with APS such as livedo reticularis (LR), thrombocytopenia, sicca-ophthalmic(sicca), heart, or neurological manifestations are not included in the APS clinical classification criteria. Circulating immune complexes (CIC) formed by Beta-2-glycoprotein I (B2GPI) and aPL (B2-CIC) have been described and their presence has been related with thrombotic events...
2018: Frontiers in Immunology
Jiang Cao, Changxiao Zhang, Xiao Han, Hai Cheng, Wei Chen, Kunming Qi, Jianlin Qiao, Zengtian Sun, Qingyun Wu, Lingyu Zeng, Mingshan Niu, Li Li, Kailin Xu
Immune thrombocytopenia (ITP) is an acquired autoimmune disease characterized by autoantibody-mediated platelet destruction. Multiple factors have been implicated in ITP pathogenesis, including T-lymphocyte dysfunctions. Increasing studies have indicated that stem cell memory-like T cell (TSCM) plays an important role in the development of multiple autoimmune diseases. This study aimed to explore the clinical correlation between the TSCM subset and ITP. The percentages of peripheral blood naïve T cells (TNs), TSCMs, central memory T cells (TCMs), effector memory T cells (TEMs), and effector T cells (TEs) among CD4+ and CD8+ T cells in 20 ITP patients before and after treatment were detected using flow cytometry...
December 1, 2018: Scandinavian Journal of Immunology
Hao Gu, Run-Hui Wu
Immune-mediated hemocytopenia is a common cytopenic diseases without bone marrow hematopoietic abnormalities, the patient's quality of life is significantly reduced when first-line treatments are ineffective. Rapamycin, possesses a clear mechanism of targeting mTOR protein, can upregulate regulatory T cells and induces apoptosis of specific cells, by regulating the lymphocyte subsets, so as to treat various types of immune-mediated hemocytopenia with a certain therapeutic effect. In this reviews, the action mechanism and clinical application of rapamycin in immune thrombocytopenia(ITP), autoimmune hemolytic anemia(AIHA), acquired aplastic anemia and autoimmune lymphoproliferative syndrome(ALPS) etc...
December 2018: Zhongguo Shi Yan Xue Ye Xue za Zhi
Emine E Yalinbaş, Melike Sezgin Evim, Özcan Bör, Zafer Gülbaş
OBJECTIVE: Immune thrombocytopenia (ITP) is an autoimmune disease, and it has become evident that T lymphocytes play an important role in the pathogenesis of ITP. We investigated the role of T helper (Th) intracellular IL-2, IL-4, IL-6, IFN-γ, and T lymphocyte apoptosis in the pathogenesis of acute ITP and the effect of glucocorticoid treatment on cytokine profile. We investigated also P-glycoprotein (P-gp) and glucocorticoid receptor (GCR) expression as a possible mechanism for glucocorticoid resistance...
November 28, 2018: Journal of Pediatric Hematology/oncology
Garg Ambar, Arya Vandana, Duggal Lalit, Kotwal Jyoti
Background: For many years, azathioprine and its active metabolite 6-merceptopurine are used as immunosuppressants for treatment of autoimmune disorders. However, azathioprine has low therapeutic index with myelosuppression as its predominant toxicity which is linked with thiopurine S-methyltransferase (TPMT) enzyme activity, which is involved in drug metabolism. TPMT activity is controlled by variants in TPMT gene. We aimed to estimate prevalence of TPMT gene mutations in North Indian patients with autoimmune disorders and to assess myelosuppression in these patients...
May 2018: Journal of the Association of Physicians of India
Guangyu Wei, Qi Luo, Xiamin Wang, Xiaoqing Wu, Mengdi Xu, Ning Ding, Yan Zhao, Lamei Zhong, Jurui Wang, Yulu Wu, Xiaoqian Li, Yun Liu, Wen Ju, Zhenyu Li, Lingyu Zeng, Kailin Xu, Jianlin Qiao
Immune thrombocytopenia (ITP) is a heterogeneous autoimmune disease, characterized by accelerated platelet destruction/clearance or decreased platelet production. ADAM17-mediated platelet receptor GPIbα extracellular domain shedding has been shown to be involved in platelet clearance. Whether GPIbα shedding participates in the pathogenesis of ITP remains poorly understood. This study aims to investigate the role of GPIbα shedding in the development of ITP via incubating normal platelets with ITP plasma to mimic ITP in vivo environment...
November 13, 2018: International Immunopharmacology
Rurong Sun, Weiying Gu, Yingchun Ma, Jing Wang, Min Wu
RATIONALE: Thrombotic thrombocytopenic purpura (TTP) is a rare, fatal disorder which could be caused by autoimmune diseases. However, TTP secondary to Sjögren syndrome (SS) is extremely rare. PATIENT CONCERNS: A 47-year- old woman with an 8-year history of SS was admitted due to skin ecchymosis and bleeding gums. Then she gradually developed fever and headache. DIAGNOSES: Laboratory investigations suggested anemia, thrombocytopenia, increased lactic dehydrogenase, and a disintegrin-like metalloproteinase with thrombospondin motif type 1 member 13 (ADAMTS13) activity deficiency with high inhibitor titers...
October 2018: Medicine (Baltimore)
Takaaki Maekawa, Yukiko Osawa, Yosuke Okada, Noriaki Tachi, Masahiro Teramoto, Toshikuni Kawamura, Toshikatsu Horiuchi, Shoichiro Kato, Ayako Kobayashi, Shinichi Kobayashi, Ken Sato, Fumihiko Kimura
Mucosal-associated invariant T (MAIT) cells help protect against certain infections and are related to some autoimmune diseases. Immune thrombocytopenia (ITP) is a relatively rare hematological autoimmune disease associated with low platelet count. We designed a cross-sectional study wherein we examined peripheral blood samples of patients with ITP for the number of MAIT cells (CD3+TCR-Vα7.2+CD161+IL-18Rα+ lymphocytes) and their CD4/8 subsets (by flow cytometry) and levels of cytokines (by multiplex assays)...
2018: PloS One
Christine W Bruggeman, Gillian Dekkers, Remco Visser, Naneth W M Goes, Timo K van den Berg, Theo Rispens, Gestur Vidarsson, Taco W Kuijpers
Intravenous immunoglobulins (IVIg) are used in the treatment of different autoimmune and inflammatory diseases, such as immune thrombocytopenia and hemolytic anemia. One of the modes of action of IVIg is preventing phagocytosis of autoantibody-opsonized blood cells by saturation of the Fc-gamma receptors of macrophages in spleen and liver. IgG contains a fixed glycan, which is in most cases biantennary, at the asparagine residue at position 297 in the Fc tail. This glycan consists of a core structure of N-acetyl glucosamine (GlcNAc) and mannose groups, variably extended with core fucose, bisecting GlcNAc as well as terminal galactose and sialic acid...
2018: Frontiers in Immunology
Zeineb Zian, Mouna Maamar, Mohamed El Aouni, Amina Barakat, Naima Ghailani Nourouti, Rajae El Aouad, Naima Arji, Mohcine Bennani Mechita
Systemic Lupus Erythematosus (SLE) is a complex autoimmune disease with a high female predominance. To date, studies about SLE in Morocco are few. This retrospective study describes the clinical and immunological features in a series of 50 SLE Moroccan patients in University Hospital Center of Rabat, Morocco, between December 2011 and December 2013. All patients were screened for antinuclear antibodies (ANA) and anti-DNA antibodies by indirect immunofluorescence, followed by identification of anti-extractable nuclear antigen antibodies by ELISA...
2018: BioMed Research International
Mohammad Zeya Ansari, R Tolstoy, G Jagadeeswaran
Thrombocytopenia is a common complication in patients with chronic liver disease and is multi-factorial. It complicates the management and worsens the prognosis. Treatment options are costly and include platelet transfusion, splenectomy, splenic artery embolization, TIPPS and thrombopoietin (TPO) agonists. Here we are presenting a patient with decompensated liver disease with known chronic alcoholism and profound thrombocytopenia despite multiple platelet transfusions. On further work up we found a coexistent autoimmune etiology...
March 2018: Journal of the Association of Physicians of India
Julie Pell, Rosemary Greenwood, Jenny Ingram, Katherine Wale, Ian Thomas, Rebecca Kandiyali, Andrew Mumford, Andrew Dick, Catherine Bagot, Nichola Cooper, Quentin Hill, Charlotte Ann Bradbury
INTRODUCTION: Immune thrombocytopenia (ITP) is an autoimmune condition that may cause thrombocytopenia-related bleeding. Current first-line ITP treatment is with high-dose corticosteroids but frequent side effects, heterogeneous responses and high relapse rates are significant problems with only 20% remaining in sustained remission with this approach. Mycophenolate mofetil (MMF) is often used as the next treatment with efficacy in 50%-80% of patients and good tolerability but can take up to 2 months to work...
October 18, 2018: BMJ Open
Andris Skride, Matiss Sablinskis, Kristaps Sablinskis, Sandra Lejniece, Aivars Lejnieks, Walter Klepetko, Irene M Lang
BACKGROUND Antiphospholipid syndrome is an autoimmune disorder characterized by a hypercoagulable state associated with circulating antiphospholipid antibodies. The presence of antiphospholipid antibodies can result in a variety of clinical symptoms, such as thrombocytopenia, stillbirth, endocardial pathologies, and recurrent pulmonary embolism. CASE REPORT We present the case of a 23-year-old man with antiphospholipid syndrome and chronic thromboembolic pulmonary hypertension who developed severe thrombocytopenia...
October 19, 2018: American Journal of Case Reports
Mengxing Chen, Rong Yan, Kangxi Zhou, Xiaodong Li, Yang Zhang, Chunliang Liu, Mengxiao Jiang, Honglei Ye, Xingjun Meng, Ningbo Pang, Lili Zhao, Jun Liu, Weiling Xiao, Renping Hu, Qingya Cui, Wei Zhong, Yunxiao Zhao, Mingqing Zhu, Anning Lin, Changgeng Ruan, Kesheng Dai
Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by low platelet count which can cause fatal hemorrhage. ITP patients with antiplatelet glycoprotein (GP) Ib-IX autoantibodies appear refractory to conventional treatments, and the mechanism remains elusive. Here we show that the platelets undergo apoptosis in ITP patients with anti-GPIbα autoantibodies. Consistent with these findings, the anti-GPIbα monoclonal antibodies AN51 and SZ2 induce platelet apoptosis in vitro. We demonstrate that anti-GPIbα antibody binding activates Akt, which elicits platelet apoptosis through activation of phosphodiesterase (PDE3A) and PDE3A-mediated PKA inhibition...
November 6, 2018: Proceedings of the National Academy of Sciences of the United States of America
Liqiong Yao, Bei Liu, Li Jiang, Lanxia Zhou, Xiaoju Liu
OBJECTIVES: To investigate the association of cytotoxic T lymphocyte-associated antigen 4 (CTLA4) with immune thrombocytopenia (ITP). METHODS: A case-control association analysis of 277 Chinese Han children was performed. The tagging variants rs11571315 and rs3087243 in the CTLA4 gene were detected using polymerase chain reaction-restriction fragment length polymorphism method. The expression quantitative trait loci (eQTL) analysis and quantitative real-time polymerase chain reaction were performed to determine the relationship of CTLA4 with ITP...
October 14, 2018: Hematology (Amsterdam, Netherlands)
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