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Immune thrombocytopenia

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https://www.readbyqxmd.com/read/29337416/the-role-of-adamts-13-in-the-coagulopathy-of-sepsis
#1
REVIEW
Marcel Levi, Marie Scully, Mervyn Singer
The interaction between platelets and the vessel wall is mediated by various receptors and adhesive proteins, of which von Willebrand factor is most prominent. The multimeric size of von Willebrand factor is an important determinant of a more intense platelet-vessel wall interaction. and is regulated by the von Willebrand factor cleaving protease, ADAMTS-13. A deficiency in ADAMTS-13 leads to higher concentrations of ultra-large von Willebrand factor multimers and pathological platelet-vessel wall interactions, in its most typical and extreme form leading to thrombocytopenic thrombotic purpura (TTP), a thrombotic microangiopathy characterized by thrombocytopenia, non-immune haemolysis, and organ dysfunction...
January 16, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29334536/nationwide-trend-analysis-of-pediatric-inpatients-with-immune-thrombocytopenia-in-the-united-states
#2
Yusuke Okubo, Atsuhiko Handa
BACKGROUND: Several studies have reported the epidemiology of immune thrombocytopenia (ITP) among children in the United States and other countries. However, recent trends in ITP among hospitalized children and hospital course remain unknown at a national level in the United States. METHOD: Hospital discharge records of patients with ITP aged 19 years and younger were obtained for the years 2003, 2006, 2009, and 2012 using the Kids' Inpatient Database. Data were weighted to estimate the annual hospitalization rates in the United States with trend analyses...
January 12, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29333980/percentages-of-pd-1-cd4-t-cells-and-pd-l1-dcs-are-increased-and-spd-1-level-is-elevated-in-patients-with-immune-thrombocytopenia
#3
Yingying Wang, Nannan Pang, Xinyou Wang, Ying Liu, Xiujuan Wang, Lei Wang, Mingling Sun, Halida Yasen, Fang Zhao, Wenxia Fan, Jianbing Ding, Xinhong Guo
The present study is to measure the expression of programmed death (PD)-1 / programmed death ligand-1 (PD-L1) negative costimulatory molecules, soluble format sPD-1 in patients with immune thrombocytopenia (ITP), and to investigate their correlation with the secretion of cytokines. A total of 35 patients with ITP were included in the present study. Twenty healthy subjects who received physical examination at our hospital were included as control group. Peripheral blood was collected from all ITP patients and healthy subjects...
January 15, 2018: Human Vaccines & Immunotherapeutics
https://www.readbyqxmd.com/read/29333402/interleukin-1b-il-1b-31-and-il-1b-511-and-interleukin-1-receptor-antagonist-il-1ra-gene-polymorphisms-in-primary-immune-thrombocytopenia
#4
Deependra Kumar Yadav, Anil Kumar Tripathi, Divya Gupta, Saurabh Shukla, Aloukick Kumar Singh, Ashutosh Kumar, Jyotsna Agarwal, K N Prasad
Background: Immune thrombocytopenia (ITP) is an immune-mediated disease caused by autoantibodies against platelets membrane glycoproteins GPIIb/IIIa and GPIb/IX. The etiology of ITP remains unclear. This study evaluated the association of polymorphisms in interleukin (IL)-1B-31, IL-1B-511, and IL-1Ra with ITP. Methods: Genotyping of IL-1B-31, IL-1B-511, and IL-1Ra was performed in 118 ITP patients and 100 controls by polymerase chain reaction restriction fragment length polymorphism and detection of variable number tandem repeats...
December 2017: Blood Research
https://www.readbyqxmd.com/read/29333401/management-of-immune-thrombocytopenia-korean-experts-recommendation-in-2017
#5
REVIEW
Jun Ho Jang, Ji Yoon Kim, Yeung-Chul Mun, Soo-Mee Bang, Yeon Jung Lim, Dong-Yeop Shin, Young Bae Choi, Ho-Young Yhim, Jong Wook Lee, Hoon Kook
Management options for patients with immune thrombocytopenia (ITP) have evolved substantially over the past decades. The American Society of Hematology published a treatment guideline for clinicians referring to the management of ITP in 2011. This evidence-based practice guideline for ITP enables the appropriate treatment of a larger proportion of patients and the maintenance of normal platelet counts. Korean authority operates a unified mandatory national health insurance system. Even though we have a uniform standard guideline enforced by insurance reimbursement, there are several unsolved issues in real practice in ITP treatment...
December 2017: Blood Research
https://www.readbyqxmd.com/read/29332223/long-term-efficacy-of-partial-splenic-embolization-for-the-treatment-of-steroid-resistant-chronic-immune-thrombocytopenia
#6
Emi Togasaki, Naomi Shimizu, Yuhei Nagao, Chika Kawajiri-Manako, Ryoh Shimizu, Nagisa Oshima-Hasegawa, Tomoya Muto, Shokichi Tsukamoto, Shio Mitsukawa, Yusuke Takeda, Naoya Mimura, Chikako Ohwada, Masahiro Takeuchi, Emiko Sakaida, Tohru Iseki, Hideyuki Yoshitomi, Masayuki Ohtsuka, Masaru Miyazaki, Chiaki Nakaseko
Thrombopoietin-receptor agonists have been recently introduced for a second-line treatment of immune thrombocytopenia (ITP). Splenectomy has tended to be avoided because of its complications, but the response rate of splenectomy is 60-80% and it has still been considered for steroid-refractory ITP. We performed partial splenic embolization (PSE) as an alternative to splenectomy. Between 1988 and 2013, 91 patients with steroid-resistant ITP underwent PSE at our hospital, and we retrospectively analyzed the efficacy and long-term outcomes of PSE...
January 13, 2018: Annals of Hematology
https://www.readbyqxmd.com/read/29330464/eltrombopag-versus-romiplostim-in-treatment-of-children-with-persistent-or-chronic-immune-thrombocytopenia-a-systematic-review-incorporating-an-indirect-comparison-meta-analysis
#7
Jiaxing Zhang, Yi Liang, Yuan Ai, Xiaosi Li, Juan Xie, Youping Li, Wenyi Zheng, Rui He
In absence of direct comparison, we conducted an indirect-comparison meta-analysis to evaluate the efficacy and safety of thrombopoietin-receptor agonists(TPO-RAs) in treatment of pediatric persistent or chronic immune thrombocytopenia(ITP). PubMed, Embase, Cochrane Library, Clinical Trials.gov, China National Knowledge Infrastructure, and Chinese Biomedical Literature Database were searched from their earliest records to May 2017. Randomized controlled trials comparing the TPO-RAs with placebo in pediatric ITP were included...
January 12, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29330115/pediatric-onset-evans-syndrome-heterogeneous-presentation-and-high-frequency-of-monogenic-disorders-including-lrba-and-ctla4-mutations
#8
Caroline Besnard, Eva Levy, Nathalie Aladjidi, Marie-Claude Stolzenberg, Aude Magerus-Chatinet, Olivier Alibeu, Patrick Nitschke, Stéphane Blanche, Olivier Hermine, Eric Jeziorski, Judith Landman-Parker, Guy Leverger, Nizar Mahlaoui, Gérard Michel, Isabelle Pellier, Felipe Suarez, Isabelle Thuret, Geneviève de Saint-Basile, Capucine Picard, Alain Fischer, Bénédicte Neven, Frédéric Rieux-Laucat, Pierre Quartier
Evans syndrome (ES) is defined by the combination of autoimmune hemolytic anemia and immune thrombocytopenia. Clinical presentation includes manifestations of immune dysregulation, found in primary immune deficiencies, autoimmune lymphoproliferative syndrome with FAS (ALPS-FAS), Cytotoxic T Lymphocyte Antigen-4 (CTLA-4) and Lipopolysaccharide-Responsive vesicle trafficking Beige-like and Anchor protein (LRBA) defects. We report the clinical history and genetic results of 18 children with ES after excluding ALPS-FAS...
January 9, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/29327472/mesenchymal-stem-cell-deficiency-influences-megakaryocytopoiesis-through-the-tnfaip3-nf-%C3%AE%C2%BAb-smad-pathway-in-patients-with-immune-thrombocytopenia
#9
Yun He, Lin-Lin Xu, Fei-Er Feng, Qian-Ming Wang, Xiao-Lu Zhu, Chen-Cong Wang, Jia-Min Zhang, Hai-Xia Fu, Lan-Ping Xu, Kai-Yan Liu, Xiao-Jun Huang, Xiao-Hui Zhang
Immune thrombocytopenia (ITP) is an autoimmune disease. Mesenchymal stem cells (MSCs) play important roles in the physiology and homeostasis of the haematopoietic system, including supporting megakaryocytic differentiation from CD34+ haematopoietic progenitor cells. Tumour necrosis factor alpha-induced protein 3 (TNFAIP3, also termed A20) plays a key role in terminating NF-κB signalling. Human genetic studies showed that the polymorphisms of the TNFAIP3 gene may contribute to ITP susceptibility. In this study, we showed a significant decrease in TNFAIP3 and increase in NF-κB/SMAD7 in ITP-MSCs...
January 12, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29327325/circulating-level-of-th17-cells-is-associated-with-sensitivity-to-glucocorticoids-in-patients-with-immune-thrombocytopenia
#10
YiChan Zhang, TingTing Ma, Xuan Zhou, JunHao Chen, Juan Li
Glucocorticoids are a widely recognized first-line therapy for immune thrombocytopenia (ITP). However, some patients are unresponsive to glucocorticoid therapy for reasons that remain unclear. Accumulating evidence suggests that CD4+ T-cell abnormalities play a crucial role in the development of ITP. In the present study, we investigated peripheral blood CD4+ T cells, Th17-associated cytokines, and the mRNA expression level of Th17 transcription factor-RORγt-in patients with newly-diagnosed ITP before glucocorticoid therapy...
January 11, 2018: International Journal of Hematology
https://www.readbyqxmd.com/read/29326868/essential-thrombocytemia-following-immune-thrombocytopenia-with-jak2v617f-mutation
#11
Giovanni Caocci, Sandra Atzeni, Monica Usai, Giorgio La Nasa
JAK2V617F mutation is found in about 60% of cases of essential thrombocytemia (ET) and represents a driving mutation. Immune thrombocytopenia (ITP) is an autoimmune disease characterized by a low platelet (PLT) count. So far, only 2 reports described ET following ITP. For the first time we analyzed in a patient the JAK2V617F allele burden at ITP onset occurred 13 years before the ET diagnosis and found the presence of a small clone JAK2V617F positive clone (3%) raised to 27% in the following years. The association of ET and ITP could suggest similar pathogenetic mechanisms that should be further investigated...
2018: Leukemia Research Reports
https://www.readbyqxmd.com/read/29324461/efficacy-of-treatment-immune-thrombocytopenic-purpura-in-pregnancy-with-corticosteroids-and-intravenous-immunoglobulin-a-prospective-follow-up-of-suggested-practice
#12
Gisela Wegnelius, Katarina Bremme, Pelle G Lindqvist
: The current study is performed to assess a routine for treatment of immune thrombocytopenic purpura in pregnancy. A prospective programme for monitoring and treatment with intravenous immunoglobulin or cortisone in pregnancies with immune thrombocytopenic purpura was suggested to all delivery units in Sweden. Treatment should be avoided if platelet counts were more than 20 × 10/l during pregnancy with no bleeding complications and with a target of 100 × 10/l at delivery. Descriptive statistics and logistic regression analysis were used...
January 10, 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/29321413/remission-of-refractory-ascites-and-discontinuation-of-hemodialysis-after-additional-rituximab-to-long-term-glucocorticoid-therapy-in-a-patient-with-tafro-syndrome
#13
Hanako Tsurumi, Yoshihide Fujigaki, Tadashi Yamamoto, Risa Iino, Kei Taniguchi, Michito Nagura, Shigeyuki Arai, Yoshifuru Tamura, Tatsuru Ota, Shigeru Shibata, Fukuo Kondo, Nozomu Kurose, Yasufumi Masaki, Shunya Uchida
Thrombocytopenia, ascites, myelofibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome is a newly recognized but rare disease, and its treatment has not yet been established. We reported a 50-year-old woman with TAFRO syndrome diagnosed 2 years after the initial symptoms of a fever, fatigue, epigastric pain, edema, ascites, lymphadenopathy, thrombocytopenia and renal insufficiency. The patient showed refractory ascites and required hemodialysis under corticosteroid mono-therapy for suspected immune-mediated disease but was successfully treated with additive rituximab, resulting in improvement in her laboratory data, the withdrawal of hemodialysis and the disappearance of ascites...
January 11, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29314277/erasmus-syndrome-associated-with-an-immune-thrombocytopenic-purpura
#14
Maxime Fouchard, Brigitte Pan Petesch, Claire Abasq-Thomas, Alain Saraux, Laurent Misery, Emilie Brenaut
We report the observation of a 57-year-old patient followed for an Erasmus syndrome (a systemic sclerosis developing secondary to silica exposure) (1) diagnosed in 2002 after being exposed as a dental technician, for whom a severe thrombocytopenia was discovered. He suffered from a pulmonary fibrosis, polyarthralgia, esophageal dysphagia and cutaneous involvement such as finger ulcerations and sclerodactylia. The treatment was made of nifedipine 30mg/day, esomeprazole 20mg/day and bosentan 25 mg twice a day...
January 5, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29313460/thrombopoietin-and-its-receptor-expression-in-pediatric-patients-with-chronic-immune-thrombocytopenia
#15
Shanshan Li, Jingbo Shao, Min Xia, Na Zhang, Jingwei Yang, Hong Li, Hui Jiang
OBJECTIVES: Chronic immune thrombocytopenia (cITP) is common in children. However, the pathogenesis has not been fully elucidated. This study aimed to determine whether thrombopoietin (TPO) and its receptor c-mannosylation of the TPO receptor (c-Mpl) have an impact on childhood cITP. METHODS: Sixty-four patients with newly diagnosed ITP (nITP), 64 patients with persistent ITP, 80 patients with cITP, and 64 healthy children (control) were enrolled in this study. Plasma TPO was measured with an ELISA, and c-Mpl was determined by flow cytometry...
January 9, 2018: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/29308775/a-case-of-immune-thrombocytopenic-purpura-with-prolonged-aptt-time-a-clotter-hidden-in-a-bleeder
#16
M H Lee, P J Khoo, L T Gew, C F Ng
We report the case of a 23-year-old woman who presented with prolonged menstruation and multiple bruises on the limbs and trunk. Investigations revealed severe thrombocytopenia and deranged coagulation profile with markedly prolonged activated partial thromboplastin time (aPTT). Lupus anticoagulant, anti-cardiolipin antibody and anti-beta-2-glycoprotein 1 antibody were positive. She was diagnosed with Immune Thrombocytopenic Purpura (ITP) with positive antiphospholipid antibody serology and given a course of intravenous methylprednisolone and tapering doses of oral prednisolone...
December 2017: Medical Journal of Malaysia
https://www.readbyqxmd.com/read/29306729/adding-to-the-complexity-of-fetal-and-neonatal-alloimmune-thrombocytopenia-reduced-fibrinogen-binding-in-the-presence-of-anti-hpa-1a-antibody-and-hypo-responsive-neonatal-platelets
#17
E Refsum, S Meinke, G Gryfelt, A Wikman, P Höglund
BACKGROUND: In fetal and neonatal alloimmune thrombocytopenia (FNAIT), maternal alloantibodies directed against paternally-derived platelet antigens are transported across the placenta to the fetus, where they may cause thrombocytopenia. The most serious complication of FNAIT is an intracranial hemorrhage (ICH), which may cause death or life-long disability of the child. Apart from alloantibody-mediated platelet destruction, the clinical outcome in FNAIT may be affected by properties of neonatal platelets and possible functional effects on platelets caused by maternal alloantibodies...
December 29, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/29306643/drug-induced-thrombocytopenia-after-anticoagulation-with-rivaroxaban
#18
Marianne K Pop, Farhad Farokhi, Lela Iduna
A 66-year-old man presented to the emergency department with complaints of dark-colored stool and rash developing over the last couple of days. The patient was started on rivaroxaban and flecainide for months prior for atrial fibrillation. Upon arrival, he was awake, alert, and oriented with a blood pressure of 111/63mmHg, heart rate of 68 beats per minute, and oxygen saturation of 96% on room air. A review of systems was unremarkable with the exception of skin rash and light-headedness. The patient's initial laboratory results were significant for red blood cell (RBC) count of 4...
December 24, 2017: American Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29304534/platelet-function-in-itp-independent-of-platelet-count-is-consistent-over-time-and-is-associated-with-both-current-and-subsequent-bleeding-severity
#19
A L Frelinger, R F Grace, A J Gerrits, S L Carmichael, E E Forde, A D Michelson
BACKGROUND:  Treatment decisions for patients with immune thrombocytopenia (ITP) are difficult because patients with similarly low platelet counts differ in their bleeding tendency. We recently reported that platelet function tests, independent of platelet count, are associated with concurrent bleeding severity, suggesting that these tests may be useful indicators of future bleeding in ITP. OBJECTIVES:  To test this hypothesis, we evaluated the consistency of these platelet function tests over time and their association with subsequent bleeding severity...
January 2018: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29301129/amegakaryocytic-thrombocytopenia-and-subsequent-aplastic-anemia-associated-with-apparent-epstein-barr-virus-infection
#20
Ilana Levy, Ruth Laor, Nizar Jiries, Jacob Bejar, Aaron Polliack, Tamar Tadmor
Acquired amegakaryocytic thrombocytopenia (AAT), a rare entity characterized by severe thrombocytopenia and the absence of megakaryocytes in the bone marrow, may mimic or precede the diagnosis of aplastic anemia (AA). Here, we describe a patient who presented with apparent Epstein-Barr virus (EBV)-associated immune thrombocytopenia resistant to several lines of therapies, which was in fact a form of AAT with some features of AA. He eventually responded to therapy with eltrombopag, cyclosporine A (CSA), and antithymocyte globulin (ATG) and recovered completely...
January 5, 2018: Acta Haematologica
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