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Autoimmune Thrombocytopenia

Ping Zhang, Guoyang Zhang, Xiaoyan Liu, Hongyun Liu, Pengfeng Yang, Liping Ma
Immune thrombocytopenia (ITP) is a common autoimmune bleeding disorder. The breakdown of immune tolerance (regulatory T [Treg] cells and suppressor cytokines) plays an important role in ITP pathophysiology, especially in refractory ITP. Bone marrow-derived mesenchymal stem cells (BM-MSCs) show immunomodulatory properties and have been extensively utilized for autoimmune diseases. However, it has not been fully elucidated how BM-MSCs affect ITP. In this study, we explore the therapeutic mechanism of BM-MSCs on ITP in mice...
February 18, 2019: Journal of Cellular Biochemistry
Elan Mohanty, Salik Nazir, Jo-Ann I Sheppard, Daniel A Forman, Theodore E Warkentin
Spontaneous HIT syndrome is an autoimmune HIT (aHIT) disorder characterized by thrombocytopenia, thrombosis, and HIT antibodies despite no proximate heparin exposure. For unknown reasons, many cases occur after total knee arthroplasty. A 52-year-old woman presented 12 days post-total knee replacement (aspirin thromboprophylaxis) with gastrointestinal bleeding (superior mesenteric vein thrombosis); the platelet count was 63 × 109 L-1 . After bowel resection and a brief course of heparin, treatment was changed to argatroban followed by fondaparinux...
February 18, 2019: Journal of Thrombosis and Haemostasis: JTH
Di Long, Yongjian Chen, Haijing Wu, Ming Zhao, Qianjin Lu
Interleukin-21 (IL-21), an autocrine cytokine predominantly produced by follicular helper T (Tfh) and T helper 17 (Th17) cells, has been proven to play an important role in the immune system, for example, by promoting proliferation and the development of Tfh and Th17 cells, balancing helper T cell subsets, inducing B cell generation and differentiation into plasma cells, and enhancing the production of immunoglobulin. These effects are mainly mediated by activation of the JAK/STAT, MAPK and PI3K pathways...
February 14, 2019: Journal of Autoimmunity
Marie-Anne Bouldouyre, Hélène Dauphin, Nathalie Cherradou, Hélène Gros
OBJECTIVE: In the obstetric medicine movement and in response to requests for appointments in internal medicine by obstetricians and midwives, we created an internal medicine consultation within the maternity ward of our General Hospital, and provide feedback after 1 year. METHODS: This retrospective descriptive study took place at the Robert Ballanger Intercommunal Hospital Center in Aulnay-sous-Bois in Seine-Saint-Denis (France) between 3rd March 2016 and 9th March 2017, the first year of the internal medicine consultation, one afternoon every 15 days, in the maternity level 2b...
September 2018: Santé Publique: Revue Multidisciplinaire Pour la Recherche et L'action
Liang Wang, Lei Xu, Hongyuan Hao, A J Gerard Jansen, Guoqiang Liu, Honglei Li, Xinguang Liu, Yajing Zhao, Jun Peng, Ming Hou
Immune thrombocytopenia (ITP) is an autoimmune disease with a mild to severe risk of bleeding complications. First line treatment includes corticosteroids, immunoglobulins, or other. In this large cohort study, first-line strategies for treatment-naive adult primary ITP was studied in a real-world setting. Records from all adult ITP patients who received first-line treatment between January 2010 and December 2017 at Qilu Hospital were reviewed retrospectively (n = 699). During the study period, 271 patients were treated with high-dose dexamethasone (HDD) and 289 patients were treated with conventional prednisone (alone or in combination with other drugs)...
February 11, 2019: Platelets
Christine Serratrice, Timothy M Cox, Vanessa Leguy-Seguin, Elizabeth Morris, Karima Yousfi, Olivier Monnet, Annie Sibert, Wassim Allaham, Nadia Belmatoug
Type 1 Gaucher disease is a rare genetic lysosomal disorder due to acid betaglucosidase deficiency. The main features are thrombocytopenia, anemia, hepatosplenomegaly and complex skeletal disease. Complications include pulmonary hypertension, cirrhosis and splenic infarction; comorbidities, such as autoimmune phenomena, B-cell malignancies and Parkinson disease also occur. Visceral aneurysms have been only rarely noted in Gaucher disease. We report the retrospective data from patients with Gaucher disease type 1 and splenic arterial aneurysm ...
February 8, 2019: Journal of Clinical Medicine
A D Tudorancea, P L Ciurea, F A Vreju, E M Vintila, Ș C Dinescu
Dermatomyositis (DM) is part of a heterogeneous group of systemic diseases called idiopathic inflammatory myopathies. As in other autoimmune connective tissue diseases (CTD), abnormalities of hematopoietic tissue and/or peripheral blood cells may develop and represent an important prognostic factor. Most common CTD associated with thrombocytopenia (TP) are systemic lupus erythematosus and antiphospholipid syndrome. DM-related TP is less frequent and may develop in the context of an underlying malignancy. Severe TP related to myositis is a very rare occurrence...
April 2018: Current Health Sciences Journal
Tarinee Rungjirajittranon, Weerapat Owattanapanich
BACKGROUND: Immune thrombocytopenia is an acquired autoimmune disease. Recently, there has been evidence of thrombotic risk in patients with immune thrombocytopenia, but the mechanism is still inconclusive. Intravenous immunoglobulin infusion therapy is considered an efficient treatment; however, it still is associated with adverse events of fever, chills, and hypotension, as well as serious complications such as thrombosis. We report a case a patient with relapsed immune thrombocytopenia who developed ischemic stroke after an intravenous immunoglobulin infusion...
January 28, 2019: Journal of Medical Case Reports
Taylor Olmsted Kim, Amanda B Grimes, Susan Kirk, Abinaya Arulselvan, Michele Lambert, Rachael F Grace, Jenny Despotovic
Immune thrombocytopenia (ITP) is the most common autoimmune cytopenia in children. Approximately 25% of patients develop chronic disease, which may be unpredictable and challenging to treat. It is not currently possible to predict at the time of presentation which patients will have chronic disease or will experience symptoms requiring second line therapy defined as treatment beyond corticosteroids, intravenous immunoglobulin, or Rh immune globulin. A multi-institutional retrospective review of 311 pediatric patients with ITP was performed with the goal of identifying clinical characteristics associated with disease course...
January 21, 2019: American Journal of Hematology
Anum Umer, Shoaib Bhatti, Shafaq Jawed
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease affecting multiple systems by the process of inflammation and formation of auto-antibodies. When it presents in childhood, it is referred to as childhood systemic lupus erythematosus (cSLE). Cardiac tamponade is a rare but potentially lethal complication of cSLE, even rarer as an initial presentation. Sub-acute cardiac tamponade (medical tamponade) is a non-emergent type of cardiac tamponade which develops slowly over time and does not necessarily present with acute distress...
October 22, 2018: Curēus
Yajing Zhao, Panpan Han, Lei Liu, Xiaojie Wang, Pengcheng Xu, Haoyi Wang, Tianshu Yu, Yunqi Sun, Lizhen Li, Tao Sun, Xinguang Liu, Hai Zhou, Jihua Qiu, Liang Wang, Jun Peng, Shuqian Xu, Ming Hou
Immune thrombocytopenia (ITP) is an acquired autoimmune disease characterized by an immune mediated decrease in platelet number. Disturbance of CD4+ T-cell homeostasis with simultaneous decrease of CD4+ CD25+ Foxp3+ regulatory T cells (Tregs) as well as unrestricted proliferation and activation of peripheral CD4+ effector T cells underpin the pathophysiology of ITP. Indirubin is an active ingredient of a traditional Chinese herb called Indigofera tinctoria L. which is clinically used for the treatment of ITP patients...
January 4, 2019: Journal of Cellular and Molecular Medicine
Masako Tsukamoto, Katsuya Suzuki, Tsutomu Takeuchi
AIM/INTRODUCTION: Primary Sjögren's syndrome (pSS) is a prototypical systemic autoimmune disease that manifests with various signs and symptoms. Although some studies have examined these manifestations over the long-term course of the disease, the association between initial clinical and immunological factors and subsequent long-term manifestations has not been fully elucidated. The aim of this study is to identify initial clinical and immunological factors associated with subsequent manifestations in patients with pSS...
December 26, 2018: International Journal of Rheumatic Diseases
Shaoxue Ding, Chunyan Liu, Yang Li, Hui Liu, Zhaoyun Liu, Tong Chen, Tian Zhang, Zonghong Shao, Rong Fu
A type of aplastic anemia (AA), non-severe aplastic anemia (NSAA) is defined as AA that does not meet the diagnostic criteria of severe aplastic anemia (SAA). Complement component 1q (C1q) has an important role in the pathogenesis of various autoimmune diseases; however, the role of C1q in the immune pathogenesis of NSAA is not clear. The current study aimed to determine whether C1q has an important role in the pathogenesis of NSAA. Isobaric tags for relative and absolute quantitation (iTRAQ) was used to compare the protein expression in bone marrow mononuclear cells from patients with NSAA and healthy volunteers...
December 12, 2018: Molecular Medicine Reports
Zhe-Wei Zhao, Wei-Ming Kang, Zhi-Qiang Ma, Xin Ye, Jian-Chun Yu
BACKGROUND: Primary immune thrombocytopenia (ITP) is a rare autoimmune disease associated with a high bleeding risk. For those patients with gastric cancer, surgical treatment may be the only option for therapy. Here, we present the first case of gastric cancer with severe and medically refractory ITP treated by radical resection of the gastric cancer and splenectomy. CASE SUMMARY: A 54-year-old female patient was admitted to our surgical department with a 2 mo history of decreased appetite, nausea, vomiting, and weight loss, which progressed to difficulty in feeding 3 d prior to her visit...
December 6, 2018: World Journal of Clinical Cases
Yasutaka Tajima, Hiroaki Yaguchi, Yasunori Mito
To explore non-motor comorbidities of myasthenia gravis (MG), we present two cases of thymoma-associated MG patients. Alopecia, pure red cell aplasia, and thymoma- associated multiorgan autoimmunity were observed in Case 1, and alopecia, thrombocytopenia, hypogammaglobulinemia and nephrotic syndrome were observed in Case 2. In both cases, autoreactive T lymphocytes inappropriately stimulated by thymus tissue may have played key roles in generating the various autoimmune-associated symptoms. Consequently, systemic immunological involvement due to the thymoma-associated breakdown of immunoregulations in both motor and non-motor systems should be considered in MG patients...
December 18, 2018: Internal Medicine
Eswari Vilayur, Jillian de Malmanche, Paul Trevillian, David Ferreira
Thrombotic microangiopathy (TMA) after renal transplantation can be a diagnostic challenge. TMA can occur with calcineurin inhibitors, allograft rejection, infection, mutations in complement regulatory proteins and autoimmunity. A 52-year-old male renal transplant recipient presented with extensive deep vein thrombosis. He developed transfusion-dependent microangiopathic haemolytic anaemia with thrombocytopenia. He did not respond calcineurin inhibitor cessation, eculizumab or plasma exchange. ADAMTS13 and complement levels were normal...
December 13, 2018: BMJ Case Reports
Svetlan Dermendzhiev, Mariya Ivanovska, Tihomir Dermendzhiev
BACKGROUND: Allergic contact dermatitis (ACD) is common in clinical practice, but the aetiology of this disease is quite varied. A leading pathogenetic mechanism is a cell-mediated immunity. The combinations of ACD with other allergic and systemic autoimmune diseases are relatively rare, but these conditions are undoubtedly a professional challenge for practitioners. CASE REPORT: We present a case of ACD combined with other immune-allergic conditions. Aetiology and pathogenesis in these cases are not well understood...
November 25, 2018: Open Access Macedonian Journal of Medical Sciences
Urshila Durani, Ronald S Go, Neil E Kay
Autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia purpura (ITP) have been associated with B-cell lymphoproliferative disorders. Here, we review the epidemiology, pathogenesis, diagnosis, and treatment of these autoimmune disorders, specifically in the setting of B-cell malignancies. AIHA and ITP are classically associated with chronic lymphocytic leukemia (CLL) but have also been reported in plasmacytic and lymphoproliferative disorders. AIHA includes both warm AIHA and cold agglutinin disease, the latter of which is strongly associated with Waldenström macroglobulinemia...
October 2018: Clinical Advances in Hematology & Oncology: H&O
Ali Amin Asnafi, Mohammad Taha Jalali, Seyed Mohammad Sadegh Pezeshki, Kaveh Jaseb, Najmaldin Saki
BACKGROUND: Autoimmune thrombocytopenia in immune thrombocytopenic purpura (ITP), thrombotic thrombocytopenic purpura (TTP), and heparin-induced thrombocytopenia (HIT) is associated with immunologic degradation of platelets and reduced platelet counts in patients, leading to bleeding risk in patients. Considering the role of human leukocyte antigens (HLA) in the development of immune response, in this review, we examine the relationship between HLA and pathogenesis of the above-mentioned diseases...
January 4, 2019: Journal of Pediatric Hematology/oncology
Thi-Huong Nguyen, Andreas Greinacher
The polyanion heparin, which is frequently used in patients, complexes with the platelet-derived cationic chemokine platelet factor (PF4, CXCL4). This results in the formation of anti-PF4/heparin antibodies (anti-PF4/H Abs). Anti-PF4/H Abs are classified into three groups: (i) nonpathogenic Abs (group 1) with no clinical relevance; (ii) pathogenic heparin-dependent Abs (group 2), which activate platelets and can cause the severe adverse drug effect heparin-induced thrombocytopenia (HIT); and (iii) pathogenic autoimmune-HIT Abs (group 3), in which group 3 anti-PF4/H Abs causes a HIT-like autoimmune disease in the absence of heparin...
December 12, 2018: ACS Nano
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