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primary immune thrombocytopenia

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https://www.readbyqxmd.com/read/28245056/presumed-primary-immune-mediated-neutropoenia-in-35-dogs-a-retrospective-study
#1
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
#2
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
#3
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
#4
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...
February 9, 2017: Clinical Therapeutics
https://www.readbyqxmd.com/read/28160307/treatment-of-presumptive-primary-immune-mediated-thrombocytopenia-with-mycophenolate-mofetil-versus-cyclosporine-in-dogs
#5
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
#6
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
#7
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)...
January 28, 2017: Cytokine
https://www.readbyqxmd.com/read/28131440/-epidemiology-and-pharmacoepidemiology-of-immune-thrombocytopenia
#8
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
#9
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
#10
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
https://www.readbyqxmd.com/read/28096086/platelets-activate-a-pathogenic-response-to-blood-stage-plasmodium-infection-but-not-a-protective-immune-response
#11
Irene Gramaglia, Joyce Velez, Valery Combes, Georges E R Grau, Melanie Wree, Henri C van der Heyde
Clinical studies indicate that thrombocytopenia correlates with the development of severe falciparum malaria, suggesting that platelets either contribute to control of parasite replication possibly as innate parasite killer cells or function in eliciting pathogenesis. Removal of platelets by anti-CD41 mAb treatment, platelet inhibition by aspirin, and adoptive transfer of WT platelets to CD40-KO mice, which do not control parasite replication, resulted in similar parasitemia compared with control mice. Human platelets at a physiological ratio of 1 platelet to 9 RBCs did not inhibit the in vitro development or replication of blood-stage P...
January 17, 2017: Blood
https://www.readbyqxmd.com/read/28078756/infections-in-non-splenectomized-persistent-or-chronic-primary-immune-thrombocytopenia-adults-risk-factors-and-vaccination-effect
#12
G Moulis, M Lapeyre-Mestre, A Palmaro, L Sailler
Essentials The risk factors for infection in immune thrombocytopenia are not well known. We conducted a national pharmacoepidemiological study. Pulmonary disease, corticosteroids and rituximab were the main risk factors for infections. Pneumococcal and influenza vaccines were protective against infections. SUMMARY: Introduction Risk factors for infection and protective effect of vaccines in immune thrombocytopenia (ITP) patients in the era of rituximab therapy are unknown. Objectives To assess the risk factors for serious and non-serious infections (respectively, SIs and NSIs) in non-splenectomized adults treated for persistent or chronic primary ITP, including the effect of pneumococcal and influenza vaccines...
January 12, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28073042/rapid-infusions-of-human-normal-immunoglobulin-50g-l-are-safe-and-well-tolerated-in-immunodeficiencies-and-immune-thrombocytopenia
#13
Giuseppe Spadaro, Alessandra Vultaggio, A Alberto Bosi, Dietmar Reichert, Jan Janssen, Donatella Lamacchia, Liliana Nappi, Antonio Pecoraro, Cinzia Milito, Andrea Ferraro, Andrea Matucci, Francesca Bacchiarri, Valentina Carrai, Azra Hibbeler, Elisabet Speckman, Chiara Guarnieri, Serena Bongiovanni, Isabella Quinti
Intravenous immunoglobulin (IVIg) is accepted as an effective and well-tolerated treatment for primary and secondary immunodeficiencies (ID) and immune thrombocytopenia (ITP). Adverse reactions of IVIg are usually mild, comprising transient flu-like symptoms, change in blood pressure and tachycardia. However IVIg therapy can be burdensome for both patients and healthcare facilities, since the infusion may take up to 4h to administer. The objective of our multicentre, prospective, open-label phase III trial was to evaluate the tolerability and safety of human normal immunoglobulin 50g/l (Ig VENA) at high intravenous infusion rates in adult patients with ID and ITP who had previously tolerated IVIg treatment, by progressively increasing infusion rate up to 8ml/kg/hr...
January 7, 2017: International Immunopharmacology
https://www.readbyqxmd.com/read/28072956/-clinical-and-immunological-analysis-of-the-patient-with-autoimmunity-due-to-germline-stat3-gain-of-function-mutation
#14
Y Ding, Y Zhang, Y P Wang, H Y Zhao, X M Chen, X H Xue, X M Bai, Y F An, Z Y Zhang, X M Tang, X D Zhao
Objective: To investigate the clinical and immunological laboratory features and gene mutation in a female patient who carried a germline gain-of-function mutation in STAT3. Method: A patient with lymphadenopathy and pancytopenia, visited the Department of Rheumatology and Immunology of Children's Hospital of Chongqing Medical University in May 2016. The clinical and laboratory characteristics, results of immunophenotyping and exome sequencing were analyzed retrospectively and related literature was reviewed...
January 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28064294/primary-versus-secondary-immune-thrombocytopenia-in-adults-a-comparative-analysis-of-clinical-and-laboratory-attributes-in-newly-diagnosed-patients-in-southern-pakistan
#15
S Sultan, S J Ahmed, S Murad, S M Irfan
BACKGROUND: Immune thrombocytopenic purpura (ITP) is a hemorrhagic diathesis, characterized by platelets destruction alongside impaired production. Patients from Asian regions often exhibit distinctive characteristics in comparison to the western patients. We accomplished this study to evaluate the prevalence of primary versus secondary ITP along with the comparative analysis between them. The secondary objective was to determine the etiological spectrum of secondary ITP. METHODS: We illustrate the results of a large cohort of newly diagnosed adults ITP from southern Pakistan...
October 2016: Medical Journal of Malaysia
https://www.readbyqxmd.com/read/28052942/refractory-primary-immune-thrombocytopenia-with-subsequent-del-5q-mds-complete-remission-of-both-after-lenalidomide
#16
Thomas Bech Mortensen, Henrik Frederiksen, Claus Werenberg Marcher, Birgitte Preiss
A patient with refractory primary immune thrombocytopenia (ITP) characterised by severe skin and mucosal bleedings was treated with several ITP-directed therapies including cyclophosphamide. He later developed therapy-related del(5q) myelodysplastic syndrome with no dysplastic morphological features in bone marrow. He remained severely thrombocytopenic, which suggests ongoing immune mediated platelet destruction. After two 3 week cycles of low-dose lenalidomide, complete cytogenetic remission and complete normalisation of platelet count were observed...
January 4, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/27991534/efficacy-and-safety-of-thrombopoietin-receptor-agonists-in-patients-with-primary-immune-thrombocytopenia-a-systematic-review-and-meta-analysis
#17
Li Wang, Zhe Gao, Xiao-Ping Chen, Hai-Yan Zhang, Nan Yang, Fei-Yan Wang, Li-Xun Guan, Zhen-Yang Gu, Sha-Sha Zhao, Lan Luo, Hua-Ping Wei, Chun-Ji Gao
Immune thrombocytopenia (ITP) is an autoimmune disease characterized by increased platelet destruction and impaired platelet production. In this study, we conducted a systematic review and meta-analysis to determine the efficacy and safety of thrombopoietin receptor agonists (TPO-RAs) in primary ITP patients. Thirteen randomized controlled trials were included in this study, the pooled results of which demonstrated that TPO-RAs significantly increased platelet response (R) and durable response (DR) rates [risk ratio (RR): 2...
December 19, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27988971/antibody-depletion-strategy-for-the-treatment-of-suspected-antibody-mediated-rejection-in-lung-transplant-recipients-does-it-work
#18
Mary Vacha, Godefroy Chery, Amanda Hulbert, Jennifer Byrns, Clark Benedetti, Catherine Ashley Finlen Copeland, Alice Gray, Oluwatoyosi Onwuemene, Scott M Palmer, Laurie D Snyder
BACKGROUND: Donor-specific antibodies (DSAs) after lung transplantation correlate with poor outcomes. The ideal treatment strategy for antibody-mediated rejection AMR is not defined. Our institution implemented an aggressive multimodality protocol for the treatment of suspected AMR. METHODS: Lung transplant recipients with suspected AMR were treated with a standardized protocol of plasma exchange, steroids, bortezomib, rituximab, and intravenous immune globulin...
December 17, 2016: Clinical Transplantation
https://www.readbyqxmd.com/read/27981682/rituximab-in-immune-thrombocytopenia-gender-age-and-response-as-predictors-of-long-term-response
#19
Miriam Marangon, Nicola Vianelli, Francesca Palandri, Maria Gabriella Mazzucconi, Cristina Santoro, Wilma Barcellini, Bruno Fattizzo, Stefano Volpetti, Elisa Lucchini, Nicola Polverelli, Monica Carpenedo, Miriam Isola, Renato Fanin, Francesco Zaja
OBJECTIVES: To evaluate the efficacy of a salvage treatment with rituximab (RTX) in adults with primary immune thrombocytopenia (ITP), in terms of short-term response and long-term response (LTR, i.e., probability to achieve and maintain response) and to identify biological and clinical predictors of response. METHODS: We retrospectively evaluated the outcome of patients with primary ITP treated with standard dosage RTX (375 mg/m(2) × 4) as salvage therapy in five Italian centers...
December 16, 2016: European Journal of Haematology
https://www.readbyqxmd.com/read/27977588/clinical-characteristics-of-immune-thrombocytopenia-associated-with-autoimmune-disease-a-retrospective-study
#20
COMPARATIVE STUDY
Yuan Liu, Shiju Chen, Yuechi Sun, Qingyan Lin, Xining Liao, Junhui Zhang, Jiao Luo, Hongyan Qian, Lihua Duan, Guixiu Shi
To clarify clinical characteristics of immune thrombocytopenia (ITP) subsets associated with autoimmune diseases (AIDs).Five thousand five hundred twenty patients were reviewed retrospectively. One hundred four ITP patients were included for analysis. Clinical manifestations at first thrombocytopenic episode were recorded.Systemic lupus erythematosus (SLE) and primary Sjogren syndrome (pSS) accounted for a large part in AIDs associated with secondary ITP. SLE-ITP, pSS-ITP, and primary ITP (pITP) patients were different in several aspects in clinical and immunological characteristics...
December 2016: Medicine (Baltimore)
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