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primary immune thrombocytopenia

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https://www.readbyqxmd.com/read/29409398/immune-dysregulation-in-primary-immune-thrombocytopenia-patients
#1
Jiakui Zhang, Qiuye Zhang, Yingwei Li, Lili Tao, Fan Wu, Yuanyuan Shen, Qianshan Tao, Xuanxuan Xu, Can Wu, Yanjie Ruan, Jiyu Wang, Jeffrey Wang, Yiping Wang, Zhimin Zhai
OBJECTIVES: To explore the immunological abnormalities in patients with primary immune thrombocytopenia (ITP), and analyze its relationship with treatment. METHODS: Proportion of different immune cell subsets were detected in the peripheral blood of 124 ITP patients at different time points and 45 normal controls by flow cytometry. The treatments included glucocorticoids, intravenous IgG as first-line treatment and second-line drugs. RESULTS: Elevated CD4/CD8 ratio and decreased the proportion of NK and CD4 + CD25 + CD127low regulatory T cells (Tregs) were found in pre-treated ITP patients than healthy controls...
February 6, 2018: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/29402802/differential-expression-of-mir-106b-5p-and-mir-200c-3p-in-newly-diagnosed-versus-chronic-primary-immune-thrombocytopenia-patients-based-on-systematic-analysis
#2
Cheng Qian, Wenying Yan, Tengda Li, Qingya Cui, Peng Liu, Mingli Gu, Jie Guo, Weiwei Zhang, Chuanlu Ren, Tianqin Wu, Anmei Deng
BACKGROUND/AIMS: MicroRNAs (miRNAs) have been described to have important roles in primary immune thrombocytopenia (ITP). To gain additional understanding, we have now further evaluated the involvement of miRNAs in ITP. METHODS: Microarray experiments were performed to examine the expression profiles of miRNAs and mRNAs in samples from subjects with newly diagnosed ITP (G1), chronic ITP (G2), and normal controls. The systematic Pipeline of Outlier MicroRNA Analysis framework was applied to identify key miRNAs expressed in the G1 and G2 samples...
January 19, 2018: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/29382894/zika-virus-infection-elicits-auto-antibodies-to-c1q
#3
Takaaki Koma, Veljko Veljkovic, Danielle E Anderson, Lin-Fa Wang, Shannan L Rossi, Chao Shan, Pei-Yong Shi, David W Beasley, Natalya Bukreyeva, Jeanon N Smith, Steven Hallam, Cheng Huang, Veronika von Messling, Slobodan Paessler
Zika virus (ZIKV) causes mostly asymptomatic infection or mild febrile illness. However, with an increasing number of patients, various clinical features such as microcephaly, Guillain-Barré syndrome and thrombocytopenia have also been reported. To determine which host factors are related to pathogenesis, the E protein of ZIKV was analyzed with the Informational Spectrum Method, which identifies common information encoded by primary structures of the virus and the respective host protein. The data showed that the ZIKV E protein and the complement component C1q cross-spectra are characterized by a single dominant peak at the frequency F = 0...
January 30, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29360249/pre-treatment-with-standard-dose-intravenous-methylprednisolone-does-not-improve-outcomes-in-newly-diagnosed-immune-thrombocytopenia-itp
#4
Anaïs Essilini, Thibault Comont, Johanne Germain, Natacha Brun, Claire Dingremont, Brice Castel, Sophie Arista, Serge Madaule, Laurent Sailler, Maryse Lapeyre-Mestre, Odile Beyne-Rauzy, Bertrand Godeau, Daniel Adoue, Guillaume Moulis
OBJECTIVE: To assess the benefits and harms to initiate corticosteroids with intravenous methylprednisolone at a conventional dose (1 mg/kg/d) to treat adults with immune thrombocytopenia (ITP). METHODS: Population stemmed from the prospective multicenter CARMEN registry and included newly diagnosed hospitalized ITP adults with platelet counts < 30 x 109 /L. We compared the patients treated with conventional-dose methylprednisolone (CDMP) before continuing with oral prednisone to patients treated with just conventional-dose oral prednisone (CDOP)...
January 23, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29356300/pathophysiology-of-thrombotic-thrombocytopenic-purpura-and-hemolytic-uremic-syndrome
#5
Johanna A Kremer Hovinga, Silvan R Heeb, Magdalena Skowronska, Monica Schaller
Thrombotic microangiopathies are rare disorders characterized by the concomitant occurrence of severe thrombocytopenia, microangiopathic hemolytic anemia, and a variable degree of ischemic end organ damage. The latter particularly affects the brain, the heart and the kidneys. The primary forms, thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS), although in their clinical presentation often overlapping, have distinctive pathophysiologies. TTP is the consequence of a severe ADAMTS13 deficiency, immune-mediated due to circulating autoantibodies (iTTP), or caused by mutations in the ADAMTS13 gene (cTTP)...
January 22, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29355998/prevalence-and-disease-associations-in-feline-thrombocytopenia-a-retrospective-study-of-194-cases
#6
J Ellis, R Bell, D C Barnes, R Miller
OBJECTIVES: To assess the prevalence of thrombocytopenia in a referral population of cats in the UK, to identify disease processes associated with thrombocytopenia and to assess the proportion of thrombocytopenic cats that tested positive for feline leukaemia virus or feline immunodeficiency virus. MATERIALS AND METHODS: Retrospective analysis of medical records at a UK referral hospital. Cats were grouped by mechanism of thrombocytopenia and disease process (where known)...
January 22, 2018: Journal of Small Animal Practice
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
#7
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/29330464/eltrombopag-versus-romiplostim-in-treatment-of-children-with-persistent-or-chronic-immune-thrombocytopenia-a-systematic-review-incorporating-an-indirect-comparison-meta-analysis
#8
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
#9
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/29298625/initial-romiplostim-dosing-and-time-to-platelet-response-in-patients-with-treatment-refractory-immune-thrombocytopenia
#10
Ryan K DasGupta, Lauren Levine, Tracy Wiczer, Spero Cataland
Background/rationale Romiplostim is a thrombopoietin receptor agonist recommended as a second-line therapy for immune thrombocytopenia. An initial dose of 1 mcg/kg/week subcutaneously with weekly 1 mcg/kg dose escalation is recommended per package labeling. Optimizing romiplostim dosing for hospitalized, corticosteroid- and intravenous immunoglobulin-refractory patients with severe thrombocytopenia secondary to immune thrombocytopenia may be critical for improving platelet responses, reducing the risk of bleeding, and decreasing hospital length of stay...
January 1, 2018: Journal of Oncology Pharmacy Practice
https://www.readbyqxmd.com/read/29296891/misdiagnosis-of-primary-immune-thrombocytopenia-and-frequency-of-bleeding-lessons-from-the-mcmaster-itp-registry
#11
Donald M Arnold, Ishac Nazy, Rumi Clare, Anushka M Jaffer, Brandon Aubie, Na Li, John G Kelton
Nonspecific diagnostic criteria and uncertain estimates of severe bleeding events are fundamental gaps in knowledge of primary immune thrombocytopenia (ITP). To address these issues, we created the McMaster ITP Registry. In this report, we describe the methodology of the registry, the process for arriving at the diagnosis, and the frequency of bleeding. Consecutive patients with platelets <150 × 109/L from a tertiary hematology clinic in Canada were eligible. Patients completed a panel of investigations and were managed per clinical need...
November 28, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296878/management-of-newly-diagnosed-immune-thrombocytopenia-can-we-change-outcomes
#12
REVIEW
Cindy E Neunert
Immune thrombocytopenia resulting from antibody-mediated platelet destruction combined with impaired platelet production is a common cause of thrombocytopenia. The decision to treat newly diagnosed patients is based on several factors including ceasing hemorrhagic manifestations, increasing the platelet count, prevention of bleeding, and inducing remission. Current standard first-line therapy is a course of corticosteroids. Although this treatment paradigm increases the platelet count in the majority of patients, a high percentage relapse after discontinuation of corticosteroid therapy...
November 14, 2017: Blood Advances
https://www.readbyqxmd.com/read/29295846/splenectomy-for-immune-thrombocytopenia-down-but-not-out
#13
Shruti Chaturvedi, Donald M/ Arnold, Keith R McCrae
Splenectomy is an effective therapy for steroid-refractory or dependent immune thrombocytopenia (ITP). With the advent of medical alternatives such as rituximab and thrombopoietin receptor antagonists (TPO-RA), the use of splenectomy has declined and is generally reserved for patients that fail multiple medical therapies. Splenectomy removes the primary site of platelet clearance and autoantibody production, and offers the highest rate of durable response (50-70%) compared to other ITP therapies. However, there are no reliable predictors of splenectomy response, and long-term risks of infection and cardiovascular complications must be considered...
January 2, 2018: Blood
https://www.readbyqxmd.com/read/29279696/immune-thrombocytopenia-as-a-consequence-of-rocky-mountain-spotted-fever
#14
Cherisse Baldeo, Karan Seegobin, Lara Zuberi
Primary immune thrombocytopenia (ITP) - also called idiopathic thrombocytopenic purpura or immune thrombocytopenic purpura - is an acquired thrombocytopenia caused by autoantibodies against platelet antigens. It is one of the more common causes of thrombocytopenia in otherwise asymptomatic adults. Rocky Mountain spotted fever (RMSF) is a potentially lethal, but curable, tick-borne disease. We present a case of ITP that was triggered by RMSF.
September 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/29258535/lytic-viral-replication-and-immunopathology-in-a-cytomegalovirus-induced-mouse-model-of-secondary-hemophagocytic-lymphohistiocytosis
#15
Ellen Brisse, Maya Imbrechts, Tania Mitera, Jessica Vandenhaute, Carine H Wouters, Robert Snoeck, Graciela Andrei, Patrick Matthys
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare immunological disorder caused by unbridled activation of T cells and macrophages, culminating in a life-threatening cytokine storm. A genetic and acquired subtype are distinguished, termed primary and secondary HLH, respectively. Clinical manifestations of both forms are frequently preceded by a viral infection, predominantly with herpesviruses. The exact role of the viral infection in the development of the hemophagocytic syndrome remains to be further elucidated...
December 19, 2017: Virology Journal
https://www.readbyqxmd.com/read/29222284/management-of-newly-diagnosed-immune-thrombocytopenia-can-we-change-outcomes
#16
REVIEW
Cindy E Neunert
Immune thrombocytopenia resulting from antibody-mediated platelet destruction combined with impaired platelet production is a common cause of thrombocytopenia. The decision to treat newly diagnosed patients is based on several factors including ceasing hemorrhagic manifestations, increasing the platelet count, prevention of bleeding, and inducing remission. Current standard first-line therapy is a course of corticosteroids. Although this treatment paradigm increases the platelet count in the majority of patients, a high percentage relapse after discontinuation of corticosteroid therapy...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29200102/adult-primary-immune-thrombocytopenia-spleen-histology-findings-and-outcomes-according-to-rituximab-use-based-on-analysis-of-41-cases
#17
Adeline Furudoï, Étienne Rivière, Estibaliz Lazaro, Enio Furudoï, Jean-François Viallard, Marie Parrens
Immune thrombocytopenia (ITP) is an acquired antibody-mediated disease, for which splenectomy remains a curative treatment. We analyzed histology and phenotypes of ITP-splenectomy specimens from 41 adult patients, with different previous ITP-specific treatments, including B-cell-depleting rituximab (RTX) or not, in an attempt to predict splenectomy success or failure on the basis of day 56 postoperative platelet counts. RTX-naive ITP-spleen samples, compared with those from a 20-patient control trauma cohort, contained the following nonspecific, ITP-evocative, white-pulp lesions: follicular helper T-cell (programmed death-1 and inducible T-cell COStimulator) expansion in reactive follicles (P=0...
December 1, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29190168/the-effect-of-anti-thyroid-antibodies-positivity-on-children-with-primary-immune-thrombocytopenia
#18
Suzan O Mousa, Gamal T Soliman, Ahmed A-F Saedii, Emad N Hameed
Primary immune thrombocytopenia (ITP) is the most common cause of acquired thrombocytopenia in children. Anti-thyroid antibodies (aTA) have previously been found to be present in various autoimmune diseases. Our aim was to study the effect of aTA positivity (which are anti-thyroid peroxidase (aTPo) and/or anti-thyroglobulin (aTg)) on children with primary immune thrombocytopenia and their relation to treatment response. Sixty-one children with primary ITP were enrolled in the present study. They were further subdivided into: ND&P group (newly diagnosed and persistent) and chronic ITP group...
November 30, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29188582/a-low-birth-weight-infant-with-no-malformations-delivered-by-a-primary-immune-thrombocytopenia-patient-treated-with-eltrombopag
#19
Naruko Suzuki, Junji Hiraga, Yumi Hariyama, Yusuke Takagi, Haruhiko Ohashi, Yasuyuki Kishigami, Hidenori Oguchi, Yoshitoyo Kagami
Primary immune thrombocytopenia (ITP) is defined by a low platelet count secondary to antibody-mediated platelet destruction or reductions in platelet production. Although eltrombopag is a thrombopoietin receptor agonist that increases platelet production in refractory or relapsed ITP, the influence on pregnancy is limited. We present the case of a pregnant 25-year-old ITP patient referred to our hospital with a history of two induced abortions. After eradication of Helicobacter pylori and with oral prednisolone at 8 mg/day, platelet count remained below 10,000/µl...
November 29, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/29181179/efficacy-of-bendamustine-on-thrombocytopenia-and-hemolytic-anemia-secondary-to-cd5-positive-b-cell-lymphoma-with-massive-splenomegaly-in-a-patient-with-rheumatoid-arthritis
#20
Yuzuru Hosoda, Hiroshi Hagino, Norihiko Hino, Toru Motokura
Chemotherapy for lymphoma may be avoided in the presence of coincident cytopenia. In case of immune cytopenia secondary to lymphoma, treatment of cytopenia is the same for primary cases, however, chemotherapy for lymphoma may be effective at the cost of severe hematological toxicity. The present study reports a complex case of thrombocytopenia and direct antiglobulin test-negative hemolytic anemia, thus mimicking Evans syndrome, secondary to cluster of differentiation 5-positive B-cell lymphoma with massive splenomegaly, in a patient suffering from rheumatoid arthritis for two decades...
November 2017: Molecular and Clinical Oncology
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