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 Immune thrombocytopenic purpura

S Badagabettu, D M Nayak, A Kurien, V G Kamath, A Kamath, A George
INTRODUCTION: The awareness and knowledge on bleeding disorders is generally poor among the rural population. Accredited Social Health Activists (ASHAs) serve as the facilitators between the rural community and the health care system. Training of ASHAs in screening of rural population for early identification of bleeding disorders can enable prompt referral, timely detection and management of bleeding disorders. AIM: The aim of the study was to evaluate the effectiveness of an ASHA training programme for identification of suspected bleeding disorder cases...
August 13, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
Charlotte K Brierley, Sue Pavord
The autoimmune cytopenias are a group of disorders resulting primarily from autoantibody-mediated destruction of blood cells, with variable clinical sequelae depending on the severity and lineage affected. Disease presentation ranges from an asymptomatic finding on a routine full blood count to an acutely unwell patient suffering the clinical consequences of severe anaemia, neutropenia or thrombocytopenia. The cytopenia may be primary or secondary to underlying infectious, immune or malignant processes. Thrombotic thrombocytopenic purpura (TTP) is a distinct, rare but potentially life-threatening entity that classically but not invariably presents with a pentad of acute onset haemolytic anaemia, thrombocytopenia, neurological symptoms, renal impairment and fevers...
August 2018: Clinical Medicine: Journal of the Royal College of Physicians of London
Neslihan Karakurt, İlker Uslu, Canan Albayrak, Leman Tomak, Elif Ozyazici, Davut Albayrak, Canan Aygun
: A major problem associated with immune thrombocytopenic purpura (ITP) in pregnancy is neonatal thrombocytopenia. We analyzed newborns born to mothers with ITP and examined predictive factors for thrombocytopenia. This retrospective study was performed in a single academic center from January 2007 to January 2018. Pregnant women with ITP and their babies are included. All neonates had a complete blood count and cranial ultrasound (USG) performed. Twenty seven neonates of 22 mothers were evaluated. A total of 23 (85%) of neonates were thrombocytopenic (<150 × 10/l) and in 20 (74%) platelet count was below 50 × 10/l...
July 20, 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
Aneet Singh, Bhagyashri Hungund, Lalit Kumar, Mallikarjun Pattanshetti
Bicytopenia is the reduction of any of the two cell lines of blood, i.e., erythrocytes, leukocytes or platelets. Many studies are done on pancytopenia but very few studies exist in the literature evaluating the spectrum of aetiologies of bicytopenia. To date, no study is available on bicytopenia in adults. We aimed to study the clinico-haematological profile of patients with bicytopenia and to investigate the different aetiologies of bicytopenia. Four hundred patients with bicytopenia admitted to KLE Dr. Prabhakar Kore Charitable Hospital and Medical Research Centre were selected using systematic random sampling and included in the study...
August 2018: Pathology
Meera Sridharan, Ronald S Go, Maria A V Willrich
Thrombotic microangiopathies (TMA) are a class of disorders characterized by microangiopathic hemolytic anemia, non-immune thrombocytopenia, and organ dysfunction. One type of TMA is atypical hemolytic uremic syndrome (aHUS) a disorder caused by hyper-activation of the alternative complement pathway due to over activation of C3 convertases and loss of complement regulatory mechanisms. The pathophysiological mechanism of aHUS involves increased continuous spontaneous hydrolysis of C3 to C3b which leads to tissue deposition of C3b, the membrane attack complex formation and subsequent tissue injury...
July 19, 2018: Journal of Immunological Methods
Yuan-Yuan Lu, Na Guan, Qing-Hong Meng, Ming-Lei Li, Yun-Yun Liu, Ying Wang
OBJECTIVE: To explore the efficacy and safety of recombinant human thrombopoietin (rhTPO) combined with high-dose dexamethasone (DXM) in the treatment of children with refractory immune thrombocytopenic purpura (ITP). METHODS: Fifty-eight ITP children who had failed first-line therapy were randomly divided into two groups: DXM treatment (n=27) and rhTPO + DXM treatment (n=31). The DXM treatment group received two continuous cycles of DXM treatment; in each cycle, patients received high-dose DXM (0...
July 2018: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
José Carlos Jaime-Pérez, Goel Treviño-Reyna, Patrizia Aguilar-Calderón, Olga G Cantú-Rodríguez, Luis Javier Marfil-Rivera, David Gómez-Almaguer
OBJECTIVES: To demonstrate the importance of regional efforts to register features and report frequency of hematology diseases in the context of incomplete national registries. METHODS: Frequencies and salient characteristics of hematologic diseases in Northeast Mexico were documented in a reference center at a tertiary care university hospital during the decade 2005-2015. Disease categories were grouped by age, sex and diagnosis. Age group distribution followed WHO guidelines in years as children (0-17), adults (18-64) and elders (+65)...
July 16, 2018: Hematology (Amsterdam, Netherlands)
Yosuke Nishio, Yoshihiko Kawano, Jun-Ichi Kawada, Yoshinori Ito, Shinya Hara
Cytomegalovirus (CMV) is one of the major infectious etiologies that induce thrombocytopenia. Although immune thrombocytopenic purpura (ITP) in children is often preceded by viral infections, thrombocytopenia associated with active CMV infection is considered CMV-related thrombocytopenia (CMV-thrombocytopenia), which can be distinguished from ITP. CMV-thrombocytopenia is reported to be less responsive to standard therapies for ITP and may require antiviral therapies. We herein report a case of refractory CMV-thrombocytopenia that achieved complete remission without antiviral therapy...
July 9, 2018: Journal of Infection and Chemotherapy: Official Journal of the Japan Society of Chemotherapy
Matt Shirley
Avatrombopag (Doptelet® ) is an orally bioavailable, small molecule thrombopoietin receptor agonist that has been developed by Dova Pharmaceuticals for the treatment of thrombocytopenic disorders. In May 2018 avatrombopag received its first global approval, in the USA, for use in the treatment of thrombocytopenia in adult patients with chronic liver disease (CLD) who are scheduled to undergo a procedure. A Marketing Authorization Application for use of avatrombopag in this indication was submitted to the EMA in April 2018...
July 11, 2018: Drugs
N Dilay Gültekin, F Hilal Yilmaz, H Altunhan, S Findik, H Tokgöz, Ümran Çalişkan
Kasabach-Merritt syndrome is a rare life-threatening clinical presentation in neonatal period. it is characterized by giant hemangioma and serious thrombocytopenia. The diagnostic criteria include: 1) hemangiomas on skin, 2) thrombocytopenia or coagulopathy, 3) hemangioma on internal organs diagnosed by ultrasonography, computed tomography or magnetic resonance imaging, and 4) excluding reasons, such as idiopathic thrombocytopenic purpura or hypersplenism.Placental chorioangiomas are the most widespread non-trophoblastic benign tumor-like lesions of placenta...
2018: Journal of Neonatal-perinatal Medicine
Yubo Li, Yamei Li, Wenliang Lu, Hongbin Li, Yuming Wang, Houmin Luo, Yuanyuan Wu, Wenying Dong, Gang Bai, Yanjun Zhang
Current hormone-based treatments for immune thrombocytopenic purpura (ITP) are associated with potentially serious adverse reactions. Zi Dian Fang (ZDF) is a multi-target Traditional Chinese Medicine (TCM) used to treat both the symptoms and root causes of ITP, with fewer side effects than hormone-based treatments. This study analysis of the therapeutic effects of ZDF on ITP from three aspects: platelet proliferation, immunoregulation, and inflammation. After detection of 52 chemical constituents of ZDF by UPLC-Q-TOF/MS, The main targets and pathways affected by ZDF were screened by network pharmacology and verified by Western blot and ELISA...
2018: Frontiers in Pharmacology
Daniel Valente Neves, Carolina Pinto Vieira, Adriana Rocha, Vera Lucia Lanchote
PURPOSE: Ceftriaxone elimination occurs through breast cancer resistance transporter (BCRP) and multidrug resistance-associated protein 2 (MRP-2) which are expressed on the canalicular membrane of hepatocytes. Eltrombopag, a thrombopoetin receptor agonist used in the treatment of immune thrombocytopenic purpura, is reported in in vitro studies as an inhibitor of intestinal BCRP but not an inhibitor of hepatic BCRP. Thus, the present study evaluates the effect of therapeutic doses of eltrombopag on the clinical pharmacokinetics of intravenous ceftriaxone...
2018: Journal of Pharmacy & Pharmaceutical Sciences: a Publication of the Canadian Society for Pharmaceutical Sciences
Jacquelyn M Powers, Joseph R Stanek, Lakshmi Srivaths, Fareeda W Haamid, Sarah H O'Brien
STUDY OBJECTIVE: To assess the frequency, severity, and inpatient management of girls admitted with heavy menstrual bleeding and iron deficiency anemia at US children's hospitals, with a focus on hematologic considerations. DESIGN: Retrospective multicenter cohort study from October 2012 through September 2015. SETTING: Children's hospitals submitting data to the Pediatric Health Information System. PARTICIPANTS: Female patients, age 8-18 years, admitted with heavy menstrual bleeding and anemia as either a primary or secondary diagnosis...
June 22, 2018: Journal of Pediatric and Adolescent Gynecology
Masumeh Maleki Behzad, Ali Amin Asnafi, Mohammad Ali Jalalifar, Mostafa Moghtadaei, Kaveh Jaseb, Najmaldin Saki
Immune thrombocytopenic purpura (ITP) is an autoimmune bleeding disorder associated with platelet destruction. Abnormalities in frequency and function of different immune cells can play a crucial role in this disease. The aim of this study was to evaluate the prognostic value of CD markers' expressions by immune cells in ITP. Peripheral blood samples were collected from 25 ITP patients before and after treatment. The expression of CD markers was evaluated by flow cytometry technique. The expression of CD38 and CD56 was significantly lower before treatment than after it (p = 0...
June 2018: APMIS: Acta Pathologica, Microbiologica, et Immunologica Scandinavica
Mrigender S Virk, Nathan P Dean, Edward C C Wong
Current chemistry analyzers measure ion concentration using ion- selective electrodes; however, may differ in the specific technology at the bedside versus the central laboratory. Instruments utilized for point-of-care testing (POCT) at the bedside use direct ion-selective electrodes, whereas central-laboratory analyzers use indirect ion-selective electrodes. Under most circumstances, these instruments will deliver the same result; however, various substances can cause interferences in one or the other. An 18-year-old Hispanic woman with a history of immune thrombocytopenic purpura (ITP) presented at Children's National Medical Center (CNMC) with a severe headache and required intravenous immunoglobulin (IVIG) therapy...
June 12, 2018: Laboratory Medicine
H M Senanayake, M Patabendige
BACKGROUND: Thrombotic thrombocytopenic purpura and peripartum cardiomyopathy are potentially lethal complications of pregnancy. We describe a case in which both of these developed in the same patient. The etiologies of both conditions remain uncertain, but they share immune hyperreactivity as a possible cause. CASE PRESENTATION: A 33-year-old Lankan primigravida gave birth at 38 weeks of gestation by cesarean section when she presented with right-sided abdominal pain and a provisional diagnosis of appendicitis...
June 6, 2018: Journal of Medical Case Reports
Stefano Bibbò, Claudio Fozza, Giovanni Mario Pes, Rodrigo Rojas, Roberto Manetti, Maria Pina Dore
Introduction: Coeliac disease (CD) and immune thrombocytopenic purpura (ITP) are immune conditions, often associated with other immune disorders. In recent years, increasing attention has been directed towards the association between ITP and CD. Aim: To investigate the frequency of ITP in CD patients and vice versa and to assess the risk of their association. Patients and Methods: This was a prospective observational study. All consecutive patients with CD or ITP attending our department were enrolled between January 2016 and December 2017...
2018: Gastroenterology Research and Practice
R Herbrecht, M Ojeda-Uribe, D Kientz, C Fohrer, A Bohbot, O Hinschberger, K-L Liu, E Remy, C Ernst, J-S Lin, L Corash, J P Cazenave
BACKGROUND: Auto-immune thrombotic thrombocytopenic purpura (TTP) is a morbid multi-organ disorder. Cardiac involvement not recognized in initial disease descriptions is a major cause of morbidity. Therapeutic plasma exchange (TPE) requires exposure to multiple plasma donors with risk of transfusion-transmitted infection (TTI). Pathogen inactivation (PI) with amotosalen-UVA, the INTERCEPT Blood System for Plasma (IBSP) is licensed to reduce TTI risk. METHODS: An open-label, retrospective study evaluated the efficacy of quarantine plasma (QP) and IBSP in TTP and defined treatment emergent cardiac abnormalities...
May 21, 2018: Vox Sanguinis
Hiroaki Hayashi, Ryusuke Kaneko, Shunsuke Demizu, Daichi Akasaka, Manabu Tayama, Takafumi Harada, Hiroki Irie, Yoshio Ogino, Naoko Fujino, Eiji Sasaki
Spleen tyrosine kinase (Syk) is involved in regulation of B-cell receptor (BCR) and Fc receptor downstream signal pathways. Syk plays an essential role in production of inflammatory mediators and differentiation in various immune cells and is therefore an attractive target for treating inflammatory conditions, such as autoimmune and allergic diseases. We identified TAS05567 as a highly selective Syk inhibitor and evaluated its therapeutic potential in animal models. In vitro biochemical assays were performed with available kinase assay panels...
July 2018: Journal of Pharmacology and Experimental Therapeutics
Alessandro Busca, Chiara Dellacasa, Luisa Giaccone, Sara Manetta, Lucia Biale, Laura Godio, Semra Aydin, Moreno Festuccia, Lucia Brunello, Benedetto Bruno
Pure RBC aplasia (PRCS) is a well-recognized complication after allogeneic hematopoietic stem cell transplantation (HSCT). Many therapeutic options are available to treat this condition, including erythropoietin, rituximab, bortezomib, plasma exchange, immunoadsorption, donor lymphocyte infusion, mesenchymal stem cells, antithymocyte globulin, and high-dose steroids; however, treatment outcomes are often variable and can sometimes lead to disappointing results. In this brief article we report our experience with 2 patients with PRCA after major ABO-incompatible HSCT who were resistant to multiple therapeutic interventions and who eventually benefited from treatment with eltrombopag, a thrombopoietin mimetic approved by the US Food and Drug Administration for the treatment of patients with immune thrombocytopenic purpura or severe aplastic anemia refractory to immunosuppressive agents or not eligible for HSCT...
April 21, 2018: Biology of Blood and Marrow Transplantation
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