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primary immune thrombocytopenia

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https://www.readbyqxmd.com/read/28444667/clinical-significance-of-antinuclear-and-antiextractable-nuclear-antigen-antibody-in-childhood-immune-thrombocytopenia
#1
Qihui Liu, Hongzhen Xu, Xianmin Guan, Yali Shen, Xianhao Wen, Yuxia Guo, Jie Yu, Yongchun Su
This study aims to determine the clinical significance of positive antinuclear/antiextractable nuclear antigen (ANA/A-ENA) antibody on manifestation and therapeutic response of childhood immune thrombocytopenia (ITP). Overall, 1,330 patients aged between 1 and 15.6 years diagnosed with primary ITP were retrospectively analyzed, excluding those with secondary ITP. Bleeding manifestations were recorded. All patients underwent autoantibody testing and follow-up for 32 months on average (range: 23-54 months). Steroid response was also assessed...
April 25, 2017: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/28419421/upregulation-of-cd72-expression-on-cd19-cd27-memory-b%C3%A2-cells-by-cd40l-in-primary-immune-thrombocytopenia
#2
Mingen Lyu, Yating Hao, Yang Li, Cuicui Lyu, Wenjie Liu, Huiyuan Li, Feng Xue, Xiaofan Liu, Renchi Yang
CD72 is a co-receptor of B cells and regulates B cell activation. Although aberrant expression of CD72 has been reported in primary immune thrombocytopenia (ITP), it is uncertain whether this aberrant expression is restricted to specific B cell subsets. Furthermore, the mechanisms that regulate CD72 expression are unknown. In this study, we found higher frequency of CD19(+) B cells, CD19(+) CD27(+) memory B cells and lower frequency of CD19(+) CD27(-) naive B cells in active ITP patients compared with controls and patients in remission...
April 17, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28415913/characteristics-and-management-of-primary-and-other-immune-thrombocytopenias-spanish-registry-study
#3
Javier Palau, Esther Sancho, Magdalena Herrera, Sol Sánchez, María Eva Mingot, Rosa Isabel Upegui, Mª José Rodríguez Salazar, Fátima de la Cruz, Mª Cristina Fernández, Tomás José González López, José Julio Hernández, Eduardo Ríos, Mª Fernanda López-Fernández, Marta García, José-Ángel Hernández, Miguel A Sanz
BACKGROUND: The natural history and its modulation by treatments administered for immune thrombocytopenia (ITP) in the clinical practice remains unknown. In addition, little information is available on the characteristics and management of ITP in Spain. METHODS: We conducted an observational, multicenter, registry in 70 Hematology Services from Spain between 2009 and 2011, which included children from 2 months of age and adults with primary ITP or another ITP diagnosed within the last 6 months (platelet count [PC] < 100 × 10(9)/l)...
April 17, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28412855/decreased-tlr4-expression-on-monocytes-may-cause-regulatory-t-cells-abnormality-in-patients-with-primary-immune-thrombocytopenia
#4
Yating Hao, Huiyuan Li, Yang Li, Mingen Lyu, Donglei Zhang, Rongfeng Fu, Yue Guan, Shixuan Wang, Boyang Sun, Xueqing Dou, Renchi Yang
Primary immune thrombocytopenia (ITP) is an autoimmune disease with many immune dysfunctions including T helper type 1 cell (Th1) polarization and regulatory T cells (Tregs) deficiency. This study aimed to determine the effects of TLR4 on Treg differentiation and the cytokine production of peripheral blood mononuclear cells (PBMCs) from patients with ITP. We found that expression of TLR4 on monocytes was significantly decreased in patients with active ITP than that in healthy controls and it had positive correlation with platelet count...
April 17, 2017: Autoimmunity
https://www.readbyqxmd.com/read/28411254/safety-and-efficacy-of-romiplostim-in-splenectomized-and-nonsplenectomized-patients-with-primary-immune-thrombocytopenia
#5
Douglas B Cines, Jeffrey Wasser, Francesco Rodeghiero, Beng H Chong, Michael Steurer, Drew Provan, Roger Lyons, Jaime Garcia-Chavez, Nancy Carpenter, Xuena Wang, Melissa Eisen
Primary immune thrombocytopenia is an autoimmune disorder characterized by increased platelet destruction and insufficient platelet production without another identified underlying disorder. Splenectomy may alter responsiveness to treatment and/or increase the risk of thrombosis, infection, and pulmonary hypertension. This analysis evaluated the safety and efficacy of the thrombopoietin receptor agonist romiplostim in splenectomized and nonsplenectomized adults with primary immune thrombocytopenia. Data were pooled across 13 completed clinical studies in adults with immune thrombocytopenia from 2002-2014...
April 14, 2017: Haematologica
https://www.readbyqxmd.com/read/28409645/evaluation-of-platelet-function-in-thrombocytopenia
#6
Mette Tiedemann Skipper, Peter Rubak, Jesper Stentoft, Anne-Mette Hvas, Ole Halfdan Larsen
Whole blood aggregometry is a functional assay for determination of platelet function. Until now, whole blood aggregometry has not been considered feasible at low platelet counts. Hence, the objectives of the present study were to explore platelet function in thrombocytopenia using a novel index of impedance aggregometry adjusted for platelet count and evaluate the association to platelet function assessed by flow cytometry. Hirudin anticoagulated blood was collected from 20 healthy volunteers, 20 patients with primary immune thrombocytopenia (ITP), and 17 hematological cancer patients...
April 14, 2017: Platelets
https://www.readbyqxmd.com/read/28408804/spotlight-on-romiplostim-in-the-treatment-of-children-with-chronic-immune-thrombocytopenia-design-development-and-potential-place-in-therapy
#7
REVIEW
David Buchbinder, Diane Nugent, Loan Hsieh
Primary immune thrombocytopenia (ITP) is an autoimmune disorder characterized by isolated thrombocytopenia. In approximately one-third of cases, the duration of thrombocytopenia will extend beyond 12 months consistent with a diagnosis of chronic ITP. Minor bleeding manifestations are common in chronic ITP while severe or life-threatening bleeding complications are uncommon. Moreover, spontaneous resolution occurs in the majority of children with chronic ITP necessitating treatment in only those children with ongoing bleeding manifestations or impairment in health-related quality of life (HRQOL)...
2017: Drug Design, Development and Therapy
https://www.readbyqxmd.com/read/28395880/treatment-of-metastatic-uveal-melanoma-with-adoptive-transfer-of-tumour-infiltrating-lymphocytes-a-single-centre-two-stage-single-arm-phase-2-study
#8
Smita S Chandran, Robert P T Somerville, James C Yang, Richard M Sherry, Christopher A Klebanoff, Stephanie L Goff, John R Wunderlich, David N Danforth, Daniel Zlott, Biman C Paria, Arvind C Sabesan, Abhishek K Srivastava, Liqiang Xi, Trinh H Pham, Mark Raffeld, Donald E White, Mary Ann Toomey, Steven A Rosenberg, Udai S Kammula
BACKGROUND: Uveal melanoma is a rare tumour with no established treatments once metastases develop. Although a variety of immune-based therapies have shown efficacy in metastatic cutaneous melanoma, their use in ocular variants has been disappointing. Recently, adoptive T-cell therapy has shown salvage responses in multiple refractory solid tumours. Thus, we sought to determine if adoptive transfer of autologous tumour-infiltrating lymphocytes (TILs) could mediate regression of metastatic uveal melanoma...
April 7, 2017: Lancet Oncology
https://www.readbyqxmd.com/read/28395446/-tryptophan-metabolism-in-patients-with-primary-immune-thrombocytopenia-with-high-dose-of-dexamethasone
#9
Z J Li, X Q Liu, J Q Xu, Y H Liu, L M Chen, X X Chu
Objective: To test whether the tryptophan metabolism was abnormal in newly diagnosed ITP patients as well as in these patients after treatment with dexamethasone. Methods: Newly diagnosed patients with ITP between Jan 2014 and May 2015 were enrolled, including 14 females and 11 males, with a median age of 57 years and a median PLT count of 16 (0-32) ×10(9)/L. All patients were treated with oral dexamethasone. The expression levels of IDO mRNA and TTS mRNA in peripheral blood mononuclear cells (PBMC) were analyzed by real-time quantitative polymerase chain reaction...
March 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28391511/normal-and-pathological-dynamics-of-platelets-in-humans
#10
Gabriel P Langlois, Morgan Craig, Antony R Humphries, Michael C Mackey, Joseph M Mahaffy, Jacques Bélair, Thibault Moulin, Sean R Sinclair, Liangliang Wang
We develop a mathematical model of platelet, megakaryocyte, and thrombopoietin dynamics in humans. We show that there is a single stationary solution that can undergo a Hopf bifurcation, and use this information to investigate both normal and pathological platelet production, specifically cyclic thrombocytopenia. Carefully estimating model parameters from laboratory and clinical data, we then argue that a subset of parameters are involved in the genesis of cyclic thrombocytopenia based on clinical information...
April 8, 2017: Journal of Mathematical Biology
https://www.readbyqxmd.com/read/28245056/presumed-primary-immune-mediated-neutropoenia-in-35-dogs-a-retrospective-study
#11
L Devine, P J Armstrong, J C Whittemore, L Sharkey, N Bailiff, A Huang, M Rishniw
OBJECTIVES: To describe, in a cohort of dogs with presumed primary immune-mediated neutropoenia, the presenting clinical characteristics, haematology results, bone marrow characteristics, therapies used (drugs and doses), clinical response to treatment, relapse and outcome at six months and one year. METHODS: Multi-institutional recruited retrospective descriptive case series with voluntary submissions. Presumed immune-mediated neutropoenia was diagnosed based on a neutrophil concentration <1500×10(9) cells/L on a minimum of two complete blood counts, exclusion of other causes of neutropoenia based on a diagnostic bone marrow aspirate or biopsy, and exclusion of secondary immune-mediated neutropoenia...
February 28, 2017: Journal of Small Animal Practice
https://www.readbyqxmd.com/read/28219222/-relative-analysis-of-platelet-activation-with-bleeding-risk-in-patients-with-primary-immune-thrombocytopenia
#12
M E Lyu, Y Li, C C Lyu, W J Liu, Y Guan, S X Wang, R C Yang
Objective: To study the relationship between platelet activation and the degree of bleeding in patients with primary immune thrombocytopenia (ITP) . Methods: 43 patients with ITP were assessed based on ITP-BAT bleeding grading system. Platelet membrane glycoproteins (GP) Ⅰb, GPⅡb/Ⅲa and P-selectin expression were detected by flow cytometry analysis with and without adenosine diphosphate (ADP) stimulation. Association of platelet activation with platelet count, immature platelet fraction (IPF) , bleeding severity were evaluated...
January 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28203488/efficacy-and-safety-of-clairyg-%C3%A2-a-ready-to-use-intravenous-immunoglobulin-in-adult-patients-with-primary-immune-thrombocytopenia
#13
Borhane Slama, Olivier Fain, Hervé Maisonneuve, Eric Jourdan, Jean-François Viallard, Rabye Ouaja, Ousmane Alfa-Cissé, Bertrand Godeau
PURPOSE: The present study was designed to assess the efficacy and safety of IGNG that is a new liquid, saccharose and maltose-free highly purified ready-to-use 5% intravenous immunoglobulin (IVIg), in primary immune thrombocytopenic patients with severe thrombocytopenia. METHODS: Nineteen adults with a platelet count ≤ 25 × 10(9)/L received a single dose of IGNG (1 g/kg) on Day 1, with a second identical dose on Day 3 if needed. Patients were followed for 30 days...
2017: American Journal of Blood Research
https://www.readbyqxmd.com/read/28190600/cost-of-bleeding-related-episodes-in-adult-patients-with-primary-immune-thrombocytopenia-a-population-based-retrospective-cohort-study-of-administrative-claims-data-for-commercial-payers-in-the-united-states
#14
Junji Lin, Xinke Zhang, Xiaoyan Li, David Chandler, Ivy Altomare, Jeffrey S Wasser, Karynsa Cetin
PURPOSE: We estimated the real-world costs of bleeding-related episodes (BREs) in adults with primary immune thrombocytopenia (ITP). METHODS: This retrospective cohort study used the MarketScan Commercial Claims and Encounters and Medicare Supplemental and Coordination of Benefits databases. We identified adult patients diagnosed with primary ITP between 2007 and 2012, defined by at least 2 outpatient claims separated by ≥30 days or 1 inpatient claim (International Classification of Diseases, Ninth Revision, Clinical Modification diagnosis code for primary ITP [287...
March 2017: Clinical Therapeutics
https://www.readbyqxmd.com/read/28160307/treatment-of-presumptive-primary-immune-mediated-thrombocytopenia-with-mycophenolate-mofetil-versus-cyclosporine-in-dogs
#15
F O Cummings, S A Rizzo
OBJECTIVES: The objective of this study was to compare hospitalisation duration, survival times, adverse events and cost of therapy in dogs with presumptive primary immune-mediated thrombocytopenia undergoing therapy with mycophenolate mofetil and corticosteroids versus cyclosporine and corticosteroids. METHODS: A retrospective study of medical case records of dogs with presumed primary immune-mediated thrombocytopenia was conducted. Data collected included signalment, presenting complaints, haematologic and biochemical profiles, vector-borne disease testing, thoracic and abdominal radiographs, abdominal ultrasound, medications administered, duration of hospitalisation, 30- and 60-day survival, adverse events and cost of therapy...
February 2017: Journal of Small Animal Practice
https://www.readbyqxmd.com/read/28151806/goals-defining-therapy-for-primary-immune-thrombocytopenia-in-adults
#16
Lorenzo Cirasino, Stefano Semeraro
Discordances existing between the two most influential guidelines on the treatment of immune thrombocytopenia, the 2010 International Consensus Report and the 2011 America Society of Hematology guideline, continue to be reflected by the heterogeneity of clinical practice of the physicians who treat this disease. Aimed at overcoming these discordances, we hypothesized that they could be ascribed to nonshared treatment goals. We classify the indications for and goals of the various possible treatments available for adults with primary immune thrombocytopenia according to the line of treatment and the phase of disease...
February 3, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28142109/cytokine-changes-in-response-to-tpo-receptor-agonist-treatment-in-primary-immune-thrombocytopenia
#17
Ming-Ming Qu, Xue-Na Liu, Xin-Guang Liu, Qi Feng, Yang Liu, Xu Zhang, Shuang Liu, Lei Zhang, Guo-Sheng Li, Yuan-Yuan Zhu, Ming-Yun Lv, Jun Peng, Ming Hou
Thrombopoietin receptor agonists (TPO-RAs) have been clinically used in primary immune thrombocytopenia (ITP) with favorable outcomes, while their effect on cytokine regulation in ITP remains unknown. In the present study, plasma and mRNA expression levels of interleukin (IL)-2, interferon gamma (IFN-γ), IL-4, IL-17A, and transforming growth factor-β1 (TGF-β1) were determined by ELISA and real-time quantitative PCR in 26 corticosteroid-resistant/relapsed ITP patients receiving eltrombopag or rhTPO therapy and 15 healthy controls (HCs)...
April 2017: Cytokine
https://www.readbyqxmd.com/read/28131440/-epidemiology-and-pharmacoepidemiology-of-immune-thrombocytopenia
#18
G Moulis, M Lapeyre-Mestre, D Adoue, L Sailler
During the last decade, the development of large clinical and population-based cohorts led to new findings in the epidemiology and the pharmacoepidemiology of immune thrombocytopenia (ITP). The incidence is estimated to 3-4 for 10(5) inhabitants/year, with a slight female predominance and peaks in children and patients after 60 years. The incidence rate is 9 for 10(5) inhabitants/year in males after 75 years. Variations across ethnic groups are discussed. In France, there is a North-South gradient and a peak of incidence during winter suggesting the role of viruses in ITP pathophysiology...
January 25, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28110418/occurrence-of-atypical-hus-associated-with-influenza-b
#19
Karen van Hoeve, Corinne Vandermeulen, Marc Van Ranst, Elena Levtchenko, Lambert van den Heuvel, Djalila Mekahli
Hemolytic uremic syndrome (HUS) is a disease characterized by thrombotic microangiopathy with a triad of non-immune hemolytic anemia, thrombocytopenia, and renal impairment. Approximately 10% of cases of HUS are classified as atypical (aHUS). While today many genetically forms of aHUS pathology are known, only about 50% of carriers precipitate the disease. The reason remains unclear, and triggering events like intercurrent infections have been postulated. In rare cases, influenza A is the known trigger of aHUS; however, no cases of influenza B have been reported...
January 21, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28105358/primary-autoimmune-myelofibrosis-with-severe-thrombocytopenia-mimicking-immune-thrombocytopenia-a-case-report
#20
Jian Hua, Shu Matayoshi, Tomoyuki Uchida, Morihiro Inoue, Masao Hagihara
Patients presenting with bone marrow fibrosis not accompanied by well-established autoimmune diseases, such as systemic lupus erythematosus, or malignant diseases, are considered to have primary autoimmune myelofibrosis (AIMF). Primary AIMF has been reported to follow a benign course and responds well to treatment with immunosuppressive agents. Immune thrombocytopenia (ITP) is also an autoimmune disorder characterized by antiplatelet-antibody-mediated thrombocytopenia in the absence of other causes of thrombocytopenia...
December 2016: Molecular and Clinical Oncology
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