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 Immune thrombocytopenic purpura

Clara Malagón, Maria Del Pilar Gomez, Catalina Mosquera, Camilo Vargas, Tatiana Gonzalez, Cristine Arango, Lorena Martin, Pilar Perez, Laura Amaya-Uribe, Nicolas Molano-Gonzalez, Juan-Manuel Anaya
Overt polyautoimmunity (PolyA) corresponds to the presence of more than one well-defined autoimmune disease (AD) manifested clinically in a single patient. The current study aimed to describe the main characteristics of juvenile PolyA in a pediatric rheumatology setting and analyze the chronological aspects, index cases, familial autoimmunity, and clustering pattern. This was a cross-sectional and multicenter study in which 313 children with overt PolyA were included. Patients were systematically interviewed and their medical records reviewed using a questionnaire that sought information about demographic, clinical, immunological, and familial characteristics...
February 14, 2019: Autoimmunity Reviews
Andrew P Vreede, Paula L Bockenstedt, W Joseph McCune, Jason S Knight
PURPOSE OF REVIEW: Although antiphospholipid syndrome (APS) is best known for conveying increased risk of thrombotic events and pregnancy morbidity, thrombocytopenia is also recognized as a common association. In this review, we will explore the relationship between thrombocytopenia and APS, highlighting our evolving understanding - and persistent knowledge gaps - through clinically oriented questions and answers. RECENT FINDINGS: A history of thrombocytopenia likely portends a more severe APS phenotype (including increased risk of thrombosis)...
February 11, 2019: Current Opinion in Rheumatology
Ling Qi, Fei Li
Intestinal microbiome closely relates with human health and disease, which plays a critical role in the immune response, homeostasis, drug metabolism and tumorigenesis. Imbalances in the composition and function of these intestinal microbes associate with diseases. Fecal microbiota transplantation (FMT) is an established successful treatment modality for recurrent Clostridium difficile infection (CDI). The safety profile and potential therapeutic advantages of FMT for diseases associated with dysbiosis and immune dysfunction have led to many publications, mainly case series...
February 2019: Zhongguo Shi Yan Xue Ye Xue za Zhi
D Popescu, D Andronescu, P A Babes
Helicobacter Pylori (HP) persistently colonizes the stomach in about 50% of the globe population and it is the main risk factor for peptic ulcer, as well as for gastric adenocarcinoma and MALT gastric lymphoma. The treatment for HP revolutionized the management of the peptic ulcer disease, providing permanent healing in many cases. Preventing colonization of HP would be the primary prevention of gastric malignancy and peptic ulceration. At the same time, the presence of HP provides protection for some diseases (gastroesophageal reflux disease and its complications, esophageal adenocarcinoma, asthma), the eradication of the microorganism having negative repercussions...
April 2018: Current Health Sciences Journal
Xiaoyu Zhang, Jane Owens, Henrik S Olsen, Edward So, Erin Burch, Mark C McCroskey, Xianfeng Li, Gregory L Weber, Donald Bennett, Denis Rybin, Hua Zhou, Haiping Hao, Emmanuel Y Mérigeon, David S Block, Gregory LaRosa, Scott E Strome
The antiinflammatory effects of i.v. Ig (IVIG) in the treatment of autoimmune disease are due, in part, to the Fc fragments of Ig aggregates. In order to capitalize on the known antiinflammatory and tolerogenic properties of Ig Fc aggregates, we created a recombinant human IgG1 Fc multimer, GL-2045. In vitro, GL-2045 demonstrated high-avidity binding to Fc receptors, blocked the binding of circulating immune complexes from patients with rheumatoid arthritis to human Fcγ receptors (FcγRs), and inhibited antibody-mediated phagocytosis at log order-lower concentrations than IVIG...
January 24, 2019: JCI Insight
Thomas Pincez, Bénédicte Neven, Hubert Ducou Le Pointe, Pascale Varlet, Helder Fernandes, Albane Gareton, Guy Leverger, Thierry Leblanc, Hervé Chambost, Gérard Michel, Marlène Pasquet, Frédéric Millot, Olivier Hermine, Alexis Mathian, Marie Hully, Hélène Zephir, Mohamed Hamidou, Jean-Marc Durand, Yves Perel, Judith Landman-Parker, Fréderic Rieux-Laucat, Nathalie Aladjidi
PURPOSE: Immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia (AIHA) are associated in the definition of Evans syndrome (ES). The occurrence of neurological involvement in this population is poorly described and suggests an underlying primary immunodeficiency (PID). We aimed to describe the clinical manifestations, evolution, and PID profiles of these patients. METHODS: OBS'CEREVANCE is a French, nationwide prospective cohort that includes children with chronic ITP, AIHA, and ES...
January 22, 2019: Journal of Clinical Immunology
Louise Dunphy, Rhodri Williams
Gingival bleeding is a common intraoral finding, typically associated with inflamed tissues and periodontal disease. It is easily provoked by periodontal probing or toothbrushing. Spontaneous gingival bleeding rarely occurs and may be the only sign of systemic bleeding problems such as thrombocytopenia, leukaemia or coagulopathy. In pregnancy, acute onset of thrombocytopenia may occur in systemic disorders such as severe pre-eclampsia, HELLP syndrome (haemolysis, elevated liver enzymes, low platelets) or the acute fatty liver of pregnancy...
January 17, 2019: BMJ Case Reports
Paul Coppo, Adam Cuker, James N George
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy characterized by severe congenital or immune-mediated deficiency in ADAMTS13, the enzyme that cleaves von Willebrand factor multimers. This rare condition leads invariably and rapidly to a fatal outcome in the absence of treatment, and therefore raises multiple diagnostic and therapeutic challenges. The novel concepts and mechanisms identified in the laboratory for this disease have been rapidly and successfully translated into the clinic for the benefit of patients, making TTP an archetypal disease that has benefited from targeted therapies...
January 2019: Research and practice in thrombosis and haemostasis
Sophie Hügli, Anne Hügli, Federico Simonetta, Yves Chalandon
Immune Thrombocytopenic Purpura (ITP) is in rare cases secondary to solid tumors, particularly breast cancer. In these cases, the clinical course of the ITP may follow the clinical course of the primary tumor, and remission of the ITP may be induced by treatment of the primary tumor.
January 2019: Clinical Case Reports
Satoko Oka, Masaharu Nohgawa
Autoimmune diseases, including autoimmune hemolytic anemia and immune thrombocytopenic purpura, have been described in patients with non-Hodgkin lymphoma (NHL) after immunochemotherapy. However, the underlying pathogenesis remains unclear. We examined NHL patients with autoimmune cytopenia and all patients were treated with rituximab-containing therapy. The present results showed reversed imbalances in helper/suppressor T-cell populations, and an immune system imbalance may have contributed to immunological abnormalities...
January 10, 2019: Acta Haematologica
Marie Scully, Spero R Cataland, Flora Peyvandi, Paul Coppo, Paul Knöbl, Johanna A Kremer Hovinga, Ara Metjian, Javier de la Rubia, Katerina Pavenski, Filip Callewaert, Debjit Biswas, Hilde De Winter, Robert K Zeldin
BACKGROUND: In acquired thrombotic thrombocytopenic purpura (TTP), an immune-mediated deficiency of the von Willebrand factor-cleaving protease ADAMTS13 allows unrestrained adhesion of von Willebrand factor multimers to platelets and microthrombosis, which result in thrombocytopenia, hemolytic anemia, and tissue ischemia. Caplacizumab, an anti-von Willebrand factor humanized, bivalent variable-domain-only immunoglobulin fragment, inhibits interaction between von Willebrand factor multimers and platelets...
January 9, 2019: New England Journal of Medicine
Dorota Radkowiak, Michał Wysocki, Anna Zychowicz, Anna Lasek, Piotr Major, Michał Pędziwiatr, Piotr Budzyński, Anna Zub-Pokrowiecka, Andrzej Budzyński
BACKGROUND: We aimed to evaluate the outcomes of laparoscopic splenectomy (LS) in patients with immune thrombocytopenic purpura (ITP) compared with operated for other indications. METHODS: Retrospective cohort study of patients who underwent LS in 1998-2017. Group 1 consisted of 256 patients operated for ITP, and Group 2 of 231 operated for other indications. Primary outcome was procedure difficulty. Secondary was perioperative course (30 days of surgery). RESULTS: Patients in Group 1 were younger (p < ...
January 9, 2019: Acta Chirurgica Belgica
Svetlana G Khaspekova, Olga N Shustova, Nina V Golubeva, Yaroslav A Naimushin, Lyubov E Larina, Alexey V Mazurov
Newborns from mothers with immune thrombocytopenic purpura (ITP) have a risk of thrombocytopenia due to passage of maternal antiplatelet antibodies into fetal/neonatal circulation. We looked for predictors of neonatal thrombocytopenia (nTP) in pregnant women with ITP. One hundred pregnant women with platelet count <100 × 109 /l, no non-immune causes of thrombocytopenia and increased platelet associated IgG (PA-IgG) were included in the study. Thirty seven and 63 of them gave birth to babies with and without nTP, respectively (nTP+ and nTP- groups)...
January 7, 2019: Platelets
Karen M Chisholm, Christopher Denton, Sioban Keel, Amy E Geddis, Min Xu, Burton E Appel, Alan B Cantor, Mark D Fleming, Akiko Shimamura
Germline mutations in RUNX1 result in autosomal dominant familial platelet disorder with associated myeloid malignancy (FPDMM). To characterize the hematopathologic features associated with a germline RUNX1 mutation, we reviewed a total of 42 bone marrow aspirates from 14 FPDMM patients, including 24 cases with no cytogenetic clonal abnormalities, and 18 with clonal karyotypes or leukemia. We found that all aspirate smears had ≥10% atypical megakaryocytes, predominantly characterized by small forms with hypolobated and eccentric nuclei, and forms with high nuclear-to-cytoplasmic ratios...
January 2, 2019: Pediatric and Developmental Pathology
Blake Giarola, Jennifer Massey, Yael Barnett, Michael Rodrigues, Ian Sutton
Secondary autoimmune disorders are a recognised complication of alemtuzumab treatment for multiple sclerosis. We report a case of autoimmune encephalitis manifesting as a polymorphic epilepsia partialis continua / status epilepticus seven months after the second course of alemtuzumab in a patient with previous autoimmune hypothyroidism and immune thrombocytopenic purpura. An MRI revealed multifocal cortical abnormalities and neuronal loss was evident on biopsy. Although testing for anti-neuronal antibodies was negative, the patient responded well to immunotherapy including plasma exchange...
December 3, 2018: Multiple Sclerosis and related Disorders
Jing-Wen Zhang, Yu-Xuan Luo, Yan-Ling Yang, Bing Long, Ying Lu, Xiang-Zhong Zhang
BACKGROUND: X-linked thrombocytopenia (XLT) is a milder form of Wiskott-Aldrich syndrome (WAS), characterized predominantly by thrombocytopenia with small-sized platelets. Mutations in the WAS gene are responsible for the disease. We herein detected a new mutation in the WAS gene responsible for XLT in a 3-generation Chinese pedigree. METHODS: Peripheral blood samples were collected from 7 members in the family. WAS gene was amplified from genomic DNA isolated from leucocytes, and then direct sequencing was performed...
October 31, 2018: Clinical Laboratory
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
Joana Gomes de Amorim, Manuel Rocha Abecasis, Filipa Maria Nogueira Lança Rodrigues
Thrombocytopenia is the most common hemostatic change in pregnancy, but severe thrombocytopenia is rare. One of the causes, immune thrombocytopenic purpura (ITP), is characterized by increased platelet destruction by immunoglobulin G (IgG) antibodies, presenting a high risk of hemorrhage for the patient, but also for the fetus, since antibodies may cross the placenta. We present the case of a 23-year-old pregnant woman with a history of Langerhans cell histiocytosis of the mandible submitted to surgery and chemotherapy when she was 10 years old, with diagnosis of ITP since then...
December 2018: Revista Brasileira de Ginecologia e Obstetrícia
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
An-Sofie Schelpe, Elien Roose, Bérangère S Joly, Inge Pareyn, Ilaria Mancini, Marina Biganzoli, Hans Deckmyn, Jan Voorberg, Rob Fijnheer, Flora Peyvandi, Simon F De Meyer, Paul Coppo, Agnes Veyradier, Karen Vanhoorelbeke
In autoantibody-mediated autoimmune diseases, autoantibody profiling allows to stratify patients and link autoantibodies with disease severity and outcome. However, in immune-mediated thrombotic thrombocytopenic purpura patients, stratification according to antibody profiles and their clinical relevance has not been fully explored. We aimed at developing a new type of autoantibody profiling assay for immune-mediated thrombotic thrombocytopenic purpura based on the use of anti-idiotypic antibodies. Anti-idiotypic antibodies against 3 anti-spacer autoantibodies were generated in mice and were used to capture the respective anti-spacer idiotopes from 151 acute immune-mediated thrombotic thrombocytopenic purpura plasma samples...
December 6, 2018: Haematologica
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