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primary immune thrombocytopenia

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https://www.readbyqxmd.com/read/27905681/childhood-immune-thrombocytopenia-a-nationwide-cohort-study-on-condition-management-and-outcomes
#1
Lamiae Grimaldi-Bensouda, Clémentine Nordon, Thierry Leblanc, Lucien Abenhaim, Slimane Allali, Corinne Armari-Alla, Claire Berger, Mary-France Courcoux, Fanny Fouyssac, Cécile Guillaumat, Corinne Guitton, Philippe Le Moine, Françoise Mazingue, Corinne Pondarré, Caroline Thomas, Marlène Pasquet, Yves Perel, Guy Leverger, Nathalie Aladjidi
OBJECTIVES: Nationwide prospective cohort study exploring (i) the factors associated with treatment initiation (vs. watchful waiting) in children with primary immune thrombocytopenia (ITP) followed in routine clinical practice and (ii) the predictors of chronicity at 12 months. PROCEDURE: Between 2008 and 2013, 23 centers throughout France consecutively included 257 children aged 6 months-18 years and diagnosed with primary ITP over a 5-year period. Data on ITP clinical features along with medical management were collected at baseline and 12 months...
December 1, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27902998/autoimmune-cytopenias-diagnosis-management
#2
Christian P Nixon, Joseph D Sweeney
The autoimmune cytopenias are a related group of disorders in which differentiated hematopoietic cells are destroyed by the immune system. Single lineage disease is characterized by the production of autoantibodies against red cells (autoimmune hemolytic anemia [AIHA]), platelets (autoimmune thrombocytopenia [ITP]) and neutrophils (autoimmune neutropenia [AIN]) whereas multilineage disease may include various combinations of these conditions. Central to the genesis of this disease is the breakdown of central and/or peripheral tolerance, and the subsequent production of autoantibodies by both tissue and circulating self-reactive B lymphocytes with support from T helper lymphocytes...
December 1, 2016: Rhode Island Medical Journal
https://www.readbyqxmd.com/read/27902585/long-term-complications-of-splenectomy-in-adult-immune-thrombocytopenia
#3
Lan-Huong Thai, Matthieu Mahévas, Françoise Roudot-Thoraval, Nicolas Limal, Laetitia Languille, Guillaume Dumas, Mehdi Khellaf, Philippe Bierling, Marc Michel, Bertrand Godeau
The recent large decrease in splenectomy use for chronic immune thrombocytopenia (ITP) is partly due to still-unsolved questions about long-term safety. We performed the first single-center exposed/unexposed cohort study evaluating the long-term incidence of splenectomy complications in patients with primary ITP. Overall, 83 patients who underwent splenectomy more than 10 years ago (exposed) were matched with 83 nonsplenectomized patients (unexposed) on the date of ITP diagnosis ±5 years, age and gender. After a median follow-up of 192 months (range 0...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27893685/acute-liver-failure-caused-by-hemophagocytic-lymphohistiocytosis-in-adults-a-case-report-and-review-of-the-literature
#4
Shide Lin, Ying Li, Jun Long, Qichuan Liu, Fangwan Yang, Yihuai He
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare condition that can be caused by a primary or acquired disorder of uncontrolled immune response. Liver injury is a common complication of HLH; however, HLH presenting as acute liver failure (ALF) has rarely been reported in adults. CASE SUMMARY: A 34-year-old man was admitted to our hospital with nausea and fatigue persisting for 2 weeks and jaundice for 1 week. He had hyperthermia at the onset of disease...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27869591/vertebrate-host-susceptibility-to-heartland-virus
#5
Angela M Bosco-Lauth, Amanda E Calvert, J Jeffrey Root, Tom Gidlewski, Brian H Bird, Richard A Bowen, Atis Muehlenbachs, Sherif R Zaki, Aaron C Brault
Heartland virus (HRTV) is a recently described phlebovirus initially isolated in 2009 from 2 humans who had leukopenia and thrombocytopenia. Serologic assessment of domestic and wild animal populations near the residence of 1 of these persons showed high exposure rates to raccoons, white-tailed deer, and horses. To our knowledge, no laboratory-based assessments of viremic potential of animals infected with HRTV have been performed. We experimentally inoculated several vertebrates (raccoons, goats, chickens, rabbits, hamsters, C57BL/6 mice, and interferon-α/β/γ receptor-deficient [Ag129]) mice with this virus...
December 2016: Emerging Infectious Diseases
https://www.readbyqxmd.com/read/27819522/effect-of-thrombopoietin-receptor-agonists-on-circulating-cytokine-and-chemokine-levels-in-patients-with-primary-immune-thrombocytopenia-itp
#6
Sif Gudbrandsdottir, Waleed Ghanima, Claus H Nielsen, Xingmin Feng, Hans C Hasselbalch, James Bussel
BACKGROUND: Thrombopoietin-receptor-agonists (TPO-RAs) increase platelet production in Immune Thrombocytopenia (ITP) by stimulating Mpl. The effect of TPO-RAs on inflammatory cytokine production in ITP patients has not been well investigated. METHODS: Plasma samples from 48 ITP patients treated with TPO-RAs (median age 50 years (inter-quartile range; IQR 20-69), median platelet counts 24 × 10(9)/L (IQR 15-47 × 10(9)/L), 28 females) and 16 healthy controls (nine females, median age 37 years, IQR 22-51 years) were collected before and during treatment, and analyzed for a panel of cytokines and chemokines by enzyme-linked immunosorbent assay and immuno-bead-based multiplex assay...
November 7, 2016: Platelets
https://www.readbyqxmd.com/read/27795540/primary-immunodeficiency-disease-and-hematology
#7
Hirokazu Kanegane
Primary immunodeficiency disease (PID) is an inborn error of the immune system, and is characterized by not only susceptibility to infection but also frequent combination with autoimmune diseases and malignancies. PID is principally caused by a germline mutation, and some PID patients develop hematological abnormalities. In some patients, PID is associated with hemophagocytosis-induced cytopenia, neutropenia, thrombocytopenia, and autoimmune cytopenia. In addition, a subset of PID patients presented with myeloid dysplasia...
2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/27768015/hemolytic-uremic-syndrome-in-children
#8
Valentina Talarico, Monica Aloe, Alice Monzani, Roberto Miniero, Gianni Bona
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy defined by thrombocytopenia, non-immune microangiopathic hemolytic anemia and acute renal failure. HUS is typically classified into two primary types: 1) HUS due to infections, often associated with diarrhea (D+HUS, Shiga toxin-producing Escherichia Coli-HUS), with the rare exception of HUS due to a severe disseminated infection caused by Streptococcus; 2) HUS related to complement, such HUS is also known as "atypical HUS" and is not diarrhea associated (D-HUS, aHUS); but recent studies have shown other forms of HUS, that can occur in the course of systemic diseases or physiopathological conditions such as pregnancy, after transplantation or after drug assumption...
December 2016: Minerva Pediatrica
https://www.readbyqxmd.com/read/27758967/intravenous-immune-globulin-stewardship-program-at-a-tertiary-academic-medical-center
#9
Megan A Rocchio, James W Schurr, Aaron P Hussey, Paul M Szumita
BACKGROUND: In October 2010, a pharmacist-driven stewardship program was implemented at the Brigham and Women's Hospital to ensure continued adherence to the prescribing guideline, focusing on indications for intravenous immune globulin (IVIG) use and dosing per ideal body weight. OBJECTIVE: The primary objective was to describe an IVIG stewardship program at a tertiary academic medical center. METHODS: This was a prospective, observational study from January 2013 through December 2014...
October 6, 2016: Annals of Pharmacotherapy
https://www.readbyqxmd.com/read/27753191/long-term-results-of-splenectomy-in-adult-chronic-immune-thrombocytopenia
#10
Yue Guan, Shixuan Wang, Feng Xue, Xiaofan Liu, Lei Zhang, Huiyuan Li, Renchi Yang
OBJECTIVES: We performed this study in adult patients with chronic primary immune thrombocytopenia (ITP) to explore the long-term efficacy and safety of splenectomy. METHODS: Data of 174 patients who underwent splenectomy in our hospital from 1994 to 2014 were analyzed. RESULTS: After splenectomy, 126 (72.4%) patients achieved a complete response (CR) and 28 (16.1%) achieved a response (R). Thirty-two (20.8%) responders relapsed with a median time of 24 months...
October 18, 2016: European Journal of Haematology
https://www.readbyqxmd.com/read/27741009/reemergence-of-splenectomy-for-itp-second-line-treatment
#11
Charbel Chater, Louis Terriou, Alain Duhamel, David Launay, Jean P Chambon, François R Pruvot, Moshe Rogosnitzky, Philippe Zerbib
INTRODUCTION: Corticosteroids are still the standard first-line treatment for immune thrombocytopenic purpura (ITP). As second-line therapy, splenectomy and Rituximab are both recommended. The aim of our study was to compare the efficacy of Rituximab to splenectomy in persistent or chronic ITP patients. METHODS: Between January 1999 and March 2015, we retrospectively selected all consecutive patients who underwent an ITP second-line treatment: Rituximab or splenectomy...
November 2016: Annals of Surgery
https://www.readbyqxmd.com/read/27734464/multicentre-randomised-phase-iii-study-of-the-efficacy-and-safety-of-eltrombopag-in-chinese-patients-with-chronic-immune-thrombocytopenia
#12
Renchi Yang, Junmin Li, Jie Jin, Meijuan Huang, Ziqiang Yu, Xiaojun Xu, Xiaohui Zhang, Ming Hou
Eltrombopag, a thrombopoietin receptor agonist, raises platelet counts and reduces bleeding in patients with immune thrombocytopenia (ITP). In Chinese patients, eltrombopag was evaluated at an initial dose of 25 mg, vs. 50 mg for non-Asians, because the plasma exposure of eltrombopag is higher in East Asians. A multicentre, double-blind, randomised, placebo-controlled, 8-week, phase III study enrolled 155 patients with chronic, previously treated ITP. Dosage could be adjusted (25-75 mg/day) to maintain platelet counts 50-250 × 10(9) /l...
October 13, 2016: British Journal of Haematology
https://www.readbyqxmd.com/read/27719744/tolerability-and-safety-of-octagam%C3%A2-ivig-a%C3%A2-post-authorization-safety-analysis-of-four-non-interventional-phase-iv-trials%C3%A2
#13
Wolfgang Frenzel, Stefan Wietek, Tor-Einar Svae, Anette Debes, Daniel Svorc
OBJECTIVE: To evaluate the tolerability and safety of Octagam<sup>®</sup> 5% and 10% across all indications, ages, and treatment regimens, using data from four non-interventional post-authorization safety studies (PASS); this analysis was performed following changes in the preparation of raw material used to manufacture Octagam. METHODS: All four studies included in- and out-patients prescribed Octagam for treatment of their medical condition. Physicians used case report forms to document baseline demographics, Octagam treatment details, and data on the efficacy of Octagam, and recorded all adverse drug reactions (ADRs) and other safety data...
November 2016: International Journal of Clinical Pharmacology and Therapeutics
https://www.readbyqxmd.com/read/27695216/the-role-of-laparoscopy-in-the-identification-and-management-of-missing-accessory-spleens-after-primary-splenectomy-a-case-report-and-literature-review
#14
George Vaos, Elpis Mantadakis, Stefanos Gardikis, Michael Pitiakoudis
We present a 7-year-old boy with recurrent thrombocytopenia after primary laparoscopic splenectomy for immune thrombocytopenia (ITP). Imaging modalities (ultrasound, computed tomography scan, and scintigraphy) revealed two accessory spleens while the subsequent second laparoscopy revealed 11, which were successfully removed. The relevant medical literature is reviewed, and the value of laparoscopy for chronic ITP is highlighted.
October 2016: Journal of Indian Association of Pediatric Surgeons
https://www.readbyqxmd.com/read/27683652/atypical-manifestation-of-lps-responsive-beige-like-anchor-deficiency-syndrome-as-an-autoimmune-endocrine-disorder-without-enteropathy-and-immunodeficiency
#15
Shahrzad Bakhtiar, Frank Ruemmele, Fabienne Charbit-Henrion, Eva Lévy, Frédéric Rieux-Laucat, Nadine Cerf-Bensussan, Peter Bader, Ulrich Paetow
Monogenic primary immunodeficiency syndromes can affect one or more endocrine organs by autoimmunity during childhood. Clinical manifestations include type 1 diabetes mellitus, hypothyroidism, adrenal insufficiency, and vitiligo. Lipopolysaccharide (LPS)-responsive beige-like anchor protein (LRBA) deficiency was described in 2012 as a novel primary immunodeficiency, predominantly causing immune dysregulation and early onset enteropathy. We describe the heterogeneous clinical course of LRBA deficiency in two siblings, mimicking an autoimmune polyendocrine disorder in one of them in presence of the same underlying genetic mutation...
2016: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/27671102/eltrombopag-thrombopoietin-receptor-agonist-and-plasmapheresis-as-rescue-therapy-of-acute-post-renal-transplant-immune-thrombocytopenia-in-a-child-with-schimke-immuno-osseous-dysplasia-case-report
#16
Ryszard Grenda, Wioletta Jarmużek, Joanna Latoszyńska, Sylwester Prokurat, Jacek Rubik
SIOD is rare disorder related to SMARCAL1 or SMARCAL2 gene mutation, including (among other comorbidities) T-cell immunodeficiency, nephrotic syndrome, and renal failure. Up to 22% of primary patients may develop various autoimmune disorders. We report the case of 11-year-old male with SIOD, who presented ITP at 2 years after renal transplantation with decrease in platelet count (from normal) to 56 000/μL and then (gradually) to 2000/μL. There was no effect of iv. methylprednisolone/dexamethasone. As the presence of antibodies against GPIIb/IIIa, GPIb, and GPIaIIa platelet glycoproteins was confirmed, patient was given 50 g of IVIG and then was put on plasmapheresis; however, both showed poor direct effect...
September 26, 2016: Pediatric Transplantation
https://www.readbyqxmd.com/read/27670221/advances-in-the-pathophysiology-of-primary-immune-thrombocytopenia
#17
María Perera, Teresa Garrido
OBJECTIVES: Classically, immune thrombocytopenia (ITP) was thought to be caused by the destruction and insufficient production of platelets, as mediated by autoantibodies. More recently other immune mechanisms that contribute to the disease have been discovered. This review attempts to address the main unresolved questions in ITP. METHODS: We review the most current knowledge of the pathophysiology of ITP. Immunological effects of available therapies are also described...
September 27, 2016: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/27668071/laparoscopic-splenectomy-for-primary-immune-thrombocytopenia-current-status-and-challenges
#18
REVIEW
Dong Zheng, Chen-Song Huang, Shao-Bin Huang, Chao-Xu Zheng
Primary immune thrombocytopenia (ITP) is an immune-mediated disorder affecting both adults and children, characterised by bleeding complications and low platelet counts. Corticosteroids are the first-line therapy for ITP, but only 20%-40% of cases achieve a stable response. Splenectomy is the main therapy for patients failing to respond to corticosteroids for decades, and about two-thirds of patients achieve a long-lasting response. Although some new drugs are developed to treat ITP as second-line therapies in recent years, splenectomy is still the better choice with less cost and more efficiency...
September 16, 2016: World Journal of Gastrointestinal Endoscopy
https://www.readbyqxmd.com/read/27658982/high-dose-dexamethasone-compared-with-prednisone-for-previously-untreated-primary-immune-thrombocytopenia-a-systematic-review-and-meta-analysis
#19
Siraj Mithoowani, Kathleen Gregory-Miller, Jennifer Goy, Matthew C Miller, Grace Wang, Nastaran Noroozi, John G Kelton, Donald M Arnold
BACKGROUND: Whether high-dose dexamethasone has long-term efficacy and safety in previously untreated patients with immune thrombocytopenia is unclear. We did a systematic review and a meta-analysis of randomised trials to establish the effect of high-dose dexamethasone compared with prednisone for long-term platelet count response. METHODS: We searched MEDLINE, Embase, Cumulative Index of Nursing and Allied Health Literature, and the Cochrane Library Database for papers published from 1970 to July, 2016, and abstracts from American Society of Hematology annual meetings published from 2004 to 2015 for randomised trials comparing different corticosteroid regimens for patients with previously untreated immune thrombocytopenia who achieved a platelet count response...
October 2016: Lancet Haematology
https://www.readbyqxmd.com/read/27650208/management-of-primary-immune-thrombocytopenia-with-eltrombopag-in-a-patient-with-recent-acute-coronary-syndrome
#20
Nuria Bermejo, Raúl Sigüenza, Fátima Ibáñez
No abstract text is available yet for this article.
September 17, 2016: Revista Española de Cardiología
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