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Thrombocytopenia

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https://www.readbyqxmd.com/read/29678925/role-of-the-novel-endoribonuclease-slfn14-and-its-disease-causing-mutations-in-ribosomal-degradation
#1
Sarah J Fletcher, Vera P Pisareva, Abdullah Khan, Andrew Tcherepanov, Neil V Morgan, Andrey V Pisarev
Platelets are anucleate and mostly ribosome-free cells within the bloodstream, derived from megakaryocytes within bone marrow and crucial for cessation of bleeding at sites of injury. Inherited thrombocytopenias are a group of disorders characterized by a low platelet count and are frequently associated with excessive bleeding. SLFN14 is one of the most recently discovered genes linked to inherited thrombocytopenia where several heterozygous missense mutations in SLFN14 were identified to cause defective megakaryocyte maturation and platelet dysfunction...
April 20, 2018: RNA
https://www.readbyqxmd.com/read/29678479/hit-or-miss-a-comprehensive-contemporary-investigation-of-laboratory-tests-for-heparin-induced-thrombocytopenia
#2
Emmanuel J Favaloro, Georgia McCaughan, Soma Mohammed, Kun Kan Edwin Lau, Rosalie Gemmell, Lauren Cavanaugh, Dea Donikian, Mayuko Kondo, Timothy Brighton, Leonardo Pasalic
Heparin induced thrombocytopenia (HIT) is a rare but potentially fatal complication of heparin therapy, which in a proportion of patients causes platelet activation and thrombosis. Initial clinical assessment of the likelihood of HIT is facilitated by laboratory testing to confirm or exclude HIT. This prospective investigation was performed over an 18-month period, and has involved testing of over 300 test samples from over 100 consecutive patients. Clinical assessment by 4T score was supplemented by laboratory tests that comprised both immunological [lateral flow ('STiC'), chemiluminescence (AcuStar; HIT-IgG(PF4-H) ), ELISA (Asserachrom HPIA IgG)] and functional assays [SRA, platelet aggregation using whole blood ('Multiplate') and platelet rich plasma ('LTA')]...
April 17, 2018: Pathology
https://www.readbyqxmd.com/read/29678165/retrospective-analysis-of-the-impact-of-anthracycline-dose-reduction-and-chemotherapy-delays-on-the-outcomes-of-early-breast-cancer-molecular-subtypes
#3
Sigita Liutkauskiene, Saulius Grizas, Kristina Jureniene, Jorune Suipyte, Akvile Statnickaite, Elona Juozaityte
BACKGROUND: The objective of study was to determine the effect of anthracycline dose reduction and chemotherapy delays on 5-year overall survival in patients with stage I-III breast cancer, to establish the impact of molecular subtypes on the anthracycline modification effects and to analyze reasons for such chemotherapy scheme modifications. METHODS: Medical records of patients with stage I-III breast cancer were reviewed. Inclusion criteria involved stage I- III breast carcinoma; radical surgery performed and 4 courses of AC regimen (doxorubicin and cyclophosphamide), or at least 6 courses of FAC regimen (fluorouracil, doxorubicin and cyclophosphamide) completed; no neoadjuvant chemotherapy applied; no taxane group medications administered; medical records maintain comprehensive data on treatment and follow-up...
April 20, 2018: BMC Cancer
https://www.readbyqxmd.com/read/29677409/is-there-an-association-between-heparin-induced-thrombocytopenia-hit-and-autoimmune-disease
#4
Brent Klinkhammer, Michael Gruchalla
BACKGROUND: Heparin-induced thrombocytopenia (HIT) is a drug-induced, immunoglobulin G medicated autoimmune disorder associated with several negative clinical outcomes including increased morbidity, mortality, and increased medical costs. Previous studies have shown associations between comorbid autoimmune diseases, but there is little known about associations between HIT and autoimmunity. PURPOSE: To provide clinical data to suggest an association between HIT and autoimmunity...
March 2018: WMJ: Official Publication of the State Medical Society of Wisconsin
https://www.readbyqxmd.com/read/29676943/impact-of-preoperative-thrombocytopenia-on-the-outcome-after-coronary-artery-bypass-grafting
#5
Wail Nammas, Magnus Dalén, Stefano Rosato, Riccardo Gherli, Daniel Reichart, Giuseppe Gatti, Francesco Onorati, Giuseppe Faggian, Marisa De Feo, Ciro Bancone, Sidney Chocron, Sorosh Khodabandeh, Giuseppe Santarpino, Antonino S Rubino, Daniele Maselli, Saverio Nardella, Antonio Salsano, Tiziano Gherli, Francesco Nicolini, Marco Zanobini, Matteo Saccocci, Karl Bounader, Paola D'Errigo, Tuomas Kiviniemi, Eeva-Maija Kinnunen, Andrea Perrotti, Juhani Airaksinen, Giovanni Mariscalco, Vito G Ruggieri, Fausto Biancari
The impact of thrombocytopenia on postoperative bleeding and other major adverse events after cardiac surgery is unclear. This issue was investigated in a series of patients who underwent isolated coronary artery bypass grafting (CABG) from the prospective, multicenter E-CABG registry. Preoperative thrombocytopenia was defined as preoperative platelet count <150 × 109 /L and it was considered moderate-severe when preoperative platelet count was <100 × 109 /L. Multilevel mixed-effects regression analysis was performed to adjust the effect of thrombocytopenia on outcomes for baseline and operative covariates as well as for interinstitutional differences in patient-blood management...
April 20, 2018: Platelets
https://www.readbyqxmd.com/read/29675946/plasma-proteomics-identifies-a-chemokine-storm-in-idiopathic-multicentric-castleman-disease
#6
Sheila K Pierson, Aaron J Stonestrom, Dustin Shilling, Jason Ruth, Christopher S Nabel, Amrit Singh, Yue Ren, Katie Stone, Hongzhe Li, Frits van Rhee, David C Fajgenbaum
Human Herpesvirus-8 (HHV-8)-negative/idiopathic multicentric Castleman disease (iMCD) is a poorly understood disease involving polyclonal lymphoproliferation with dysmorphic germinal centers, constitutional symptoms, and multi-organ failure. Patients can experience thrombocytopenia, anasarca, reticulin fibrosis, renal dysfunction, organomegaly, and normal immunoglobulin levels, - iMCD-TAFRO. Others experience thrombocytosis, milder effusions, and hypergammaglobulinemia, -iMCD-Not Otherwise Specified (iMCD-NOS)...
April 20, 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29674195/thrombotic-microangiopathies-of-pregnancy-differential-diagnosis
#7
REVIEW
M Gupta, B B Feinberg, R M Burwick
Thrombotic microangiopathy (TMA) disorders are characterized by microangiopathic hemolytic anemia, thrombocytopenia and end-organ injury. In pregnancy and postpartum, TMA is most commonly encountered with HELLP (hemolysis, elevated liver enzymes, low platelet count syndrome) or preeclampsia with severe features, but rarely TMA is due to thrombotic thrombocytopenic purpura (TTP) or atypical hemolytic uremic syndrome (aHUS). Due to overlapping clinical and laboratory features, TTP and aHUS are often mistaken for preeclampsia or HELLP...
February 16, 2018: Pregnancy Hypertension
https://www.readbyqxmd.com/read/29673280/safety-and-efficacy-of-thrombopoeitin-mimetics-for-refractory-immune-thrombocytopenia-purpura-in-patients-with-systemic-lupus-erythematosus-or-antiphospholipid-syndrome-a-case-series
#8
A Lusa, A Carlson
Background While thrombopoeitin (TPO) agonists that act to simulate platelet production have been approved for use in steroid-refractory chronic immune thrombocytopenia purpura (ITP), there are few data on the safety and efficacy of these medications in patients with concurrent systemic lupus erythematosus (SLE) or antiphospholipid syndrome (APS). Given that these agents can increase all hematopoietic cell lineages, it is unclear if there is an increased risk for exacerbation of the underlying lymphocyte-driven autoimmune disease in this population...
January 1, 2018: Lupus
https://www.readbyqxmd.com/read/29673108/lenalidomide-versus-lenalidomide-dexamethasone-prolonged-treatment-after-second-line-lenalidomide-dexamethasone-induction-in-multiple-myeloma
#9
Johan Lund, Astrid Gruber, Birgitta Lauri, Adil Doganay Duru, Cecilie Blimark, Agneta Swedin, Markus Hansson, Karin Forsberg, Lucia Ahlberg, Conny Carlsson, Anders Waage, Peter Gimsing, Annette Juul Vangsted, Ulf Frølund, Erik Holmberg, Gösta Gahrton, Evren Alici, Mats Hardling, Ulf-Henrik Mellqvist, Hareth Nahi
Lenalidomide (Len) plus dexamethasone (Dex) is approved for the treatment of relapsed or refractory multiple myeloma (RRMM). It is possible that single-agent Len may be effective as prolonged treatment regimen in RRMM once patients demonstrate an initial response to Len+Dex induction. Patients with RRMM who responded to first-line Len+Dex in an observational study (NCT01430546) received up to 24 cycles of either Len (25 mg/day) or Len+Dex (25 mg/day and 40 mg/week) as prolonged treatment in a subsequent phase 2 clinical trial (NCT01450215)...
April 19, 2018: Cancer Medicine
https://www.readbyqxmd.com/read/29670795/pulmonary-vasculitis-and-a-horseshoe-kidney-in-noonan-syndrome
#10
Surasak Puvabanditsin, Rosanna Abellar, Adaora Madubuko, Rajeev Mehta, Lauren Walzer
We report a term male neonate with congenital myeloproliferative disorder, thrombocytopenia, a horseshoe kidney, feeding difficulty secondary to dysphagia/foregut dysmotility, and respiratory failure. Prenatal molecular genetic analysis revealed a fetus carrying c.184T>G (p.Tyr62Asp) pathogenic variant in PTPN11 . The infant eventually succumbed to respiratory failure. Bacterial and viral cultures/studies were all no growth/negative. Pulmonary capillaritis and vasculitis were noted at autopsy. This report presents a new case of Noonan syndrome with unusual associated disorders and a review of the literature...
2018: Case Reports in Pathology
https://www.readbyqxmd.com/read/29670210/phase-i-trial-of-histone-deacetylase-inhibitor-panobinostat-in-addition-to-glucocorticoids-for-primary-therapy-of-acute-graft-versus-host-disease
#11
Lia Perez, Hugo Fernandez, Pedro Horna, Marcie Riches, Frederick Locke, Teresa Field, John Powers, Eva Sahakian, Alejandro Villagra, Asmita Mishra, Brian Betts, Mohamed Kharfan-Dabaja, Francisca Beato, Leonel Ochoa-Bayona, Joseph Pidala, Claudio Anasetti
Glucocorticoids for primary therapy of acute GVHD have limited responses. A phase I/II trial tested 4 weeks of deacetylase inhibitor panobinostat started within 48 h of glucocorticoids (1 mg/kg/day prednisone or equivalent) as primary treatment for patients with either classic acute GVHD (n = 16) or acute GVHD overlapping with chronic (n = 6). Four patients received 2.5 mg/m2 IV three times a week (TIW). Subsequent to discontinuation of IV panobinostat, patients received oral doses (PO). Two patients treated with 10 mg TIW (PO level 1) had progressive GVHD, after which patients were treated with 5 mg TIW (PO level -1; n = 16); 31/41 adverse events were possibly related, including thrombocytopenia (n = 13), leukopenia (n = 7), hypercholesterolemia (n = 3), hypertriglyceridemia (n = 5), anemia (n = 1), fatigue (n = 1), and hepatobiliary disorder (n = 1)...
April 18, 2018: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/29670135/clinical-characteristics-and-risk-factors-for-overlapping-rheumatoid-arthritis-and-sj%C3%A3-gren-s-syndrome
#12
Huaxia Yang, Sainan Bian, Hua Chen, Li Wang, Lidan Zhao, Xuan Zhang, Yan Zhao, Xiaofeng Zeng, Fengchun Zhang
This study investigated the clinical characteristics and risk factors for overlapping rheumatoid arthritis and Sjögren's syndrome (RA/SS). Patients with RA/SS in Peking Union Medical College Hospital from January 2012 to January 2017 were retrospectively analysed and compared to those of sex- and age-matched RA or SS controls. Logistic regression analysis was used to identify risk factors. Altogether, 105 consecutive patients with RA/SS were enrolled. Ninety-seven (92.4%) of them were female, with a mean age of 51...
April 18, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29669948/survey-of-tick-borne-zoonotic-viruses-in-wild-deer-in-hokkaido-japan
#13
Leo Uchida, Daisuke Hayasaka, Mya Myat Ngwe Tun, Kouichi Morita, Yasukazu Muramatsu, Katsuro Hagiwara
Tick-borne encephalitis (TBE) and severe fever with thrombocytopenia syndrome (SFTS) are both tick-borne zoonotic diseases caused by TBE virus (TBEV) and SFTS phlebovirus (SFTSV). In 2016, a second domestic TBE case was reported in Hokkaido, Japan, after an absence of 23 years. We conducted IgG ELISA for TBEV and SFTSV on 314 deer (Cervus nippon yesoensis) serum samples collected from 3 places in Hokkaido. There were 7 seropositive samples for TBEV but none for SFTSV by ELISA. The specificity of the 7 positive samples was confirmed by neutralization tests against TBEV, and 5 sera showed 320 to 640 of 50% focus reduction endpoint titers...
April 19, 2018: Journal of Veterinary Medical Science
https://www.readbyqxmd.com/read/29669399/the-impact-of-systemic-lupus-erythematosus-on-the-clinical-phenotype-of-antiphospholipid-antibody-positive-patients-results-from-antiphospholipid-syndrome-alliance-for-clinical-trials-and-international-networking-aps-action-clinical-database-and-repository
#14
Ozan Unlu, Doruk Erkan, Medha Barbhaiya, Danieli Andrade, Iana Nascimento, Renata Rosa, Alessandra Banzato, Vittorio Pengo, Amaia Ugarte, Maria Gerosa, Lanlan Ji, Maria Efthymiou, D Ware Branch, Guilherme Raires de Jesus, Angela Tincani, H Michael Belmont, Paul R Fortin, Michelle Petri, Esther Rodriguez, Guillermo J Pons-Estel, Jason S Knight, Tatsuya Atsumi, Rohan Willis, Stephane Zuily, Maria G Tektonidou
OBJECTIVE: Although systemic lupus erythematosus (SLE) is the most common autoimmune disease associated with antiphospholipid antibodies (aPL), limited data exist on the impact of SLE on the clinical phenotype of aPL-positive patients. The primary objective was to compare the clinical, laboratory, and treatment characteristics of aPL-positive patients with or without SLE. METHODS: A secure web-based data capture system stores patient demographics, and aPL-related clinical and laboratory characteristics...
April 18, 2018: Arthritis Care & Research
https://www.readbyqxmd.com/read/29669341/management-and-neonatal-outcomes-of-pregnancies-with-fetal-neonatal-alloimmune-thrombocytopenia-a-single-center-retrospective-cohort-study
#15
Stefania Ronzoni, Johannes Keunen, Prakeshkumar S Shah, Edmond N Kelly, Rory Windrim, P Gareth Seaward, Greg Ryan
BACKGROUND: There is no consensus regarding the optimal antenatal treatment of fetal/neonatal alloimmune thrombocytopenia (F/NAIT). We aimed to review the fetal blood sampling (FBS)-related risk, fetal response to maternal intravenous immunoglobulin (IVIG), and cesarean section (CS) rate in pregnancies with a history of F/NAIT. METHODS: Maternal demographics, alloantibodies, pregnancy management, fetal and neonatal outcomes, and index case characteristics were collected...
April 18, 2018: Fetal Diagnosis and Therapy
https://www.readbyqxmd.com/read/29668541/cutaneous-hemorrhage-types-as-supportive-factors-for-predicting-chronic-immune-thrombocytopenia-in-children
#16
Sarit Kalfon, Haitham Hamadeh, Yaakov Schachter, Nechama Sharon
Our objective was to assess risk factors for developing chronic immune thrombocytopenia (ITP) in children. The charts of all consecutive children diagnosed with ITP between 2000 and 2015 at a single center were retrospectively reviewed, and clinical characteristics at initial presentation were analyzed. Sixty-two children were included in the study (mean age, 6.15 y); 44 (71%) were found to have acute ITP, and 18 (29%) developed chronic ITP (permanent or relapsing thrombocytopenia >12 mo). In a univariate analysis, cutaneous hemorrhages were observed significantly more in acute patients (90...
April 17, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29668536/recurrent-severe-iron-deficiency-anemia-and-thrombocytopenia-in-an-adolescent-male
#17
Lisa Giordano, Alejandro Llanos-Chea, Alexandra Monde, Ivy Mulinge, Dipti Dighe
An adolescent male presented with recurrent episodes over several years of severe iron deficiency anemia and associated severe thrombocytopenia. The anemia was secondary to chronic blood loss due to ulceration at the site of an ileocolonic anastomosis performed during infancy. We were able to demonstrate complete resolution of thrombocytopenia with the administration of iron, and without using steroids, intravenous immunoglobulin, or platelet transfusions. This is the first reported case of an individual with multiple episodes over several years of thrombocytopenia secondary to recurrent severe iron deficiency anemia, illustrating a predisposition to this complication in a unique patient...
April 17, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29668485/prolonged-activity-and-toxicity-of-sirolimus-in-a-patient-with-metastatic-renal-perivascular-epithelioid-cell-tumor-a-case-report-and-literature-review
#18
Alessandra Raimondi, Francesca Colombo, Giulia Pintarelli, Carlo Morosi, Salvatore L Renne, Anna M Frezza, Maristella Saponara, Angelo P Dei Tos, Arabella Mazzocchi, Salvatore Provenzano, Paolo G Casali, Silvia Stacchiotti
Perivascular epithelioid cell tumor (PEComa) is a family of mesenchymal tumors. Conventional chemotherapy has little activity in this disease, but case reports are available on the activity of mammalian target of rapamycin inhibitors (e.g. sirolimus and temsirolimus). Pharmacokinetic assays of sirolimus are available as this drug has a precise therapeutic window and blood levels might be influenced by CYP3A4 polymorphisms and drug interactions. We report on a case of a patient with metastatic, progressive PEComa who started sirolimus at a dose of 5 mg/day with evidence of grade (G) 3 mucositis, G2 thrombocytopenia, and G1 leucopenia 10 days after the treatment started, in absence of concomitant medications or prohibited food assumption...
April 17, 2018: Anti-cancer Drugs
https://www.readbyqxmd.com/read/29668136/-a-fatal-case-series-of-rocky-mountain-spotted-fever-in-sonora-m%C3%A3-xico
#19
Jesús Delgado-De la Mora, Jesús David Licona-Enríquez, Marcia Leyva-Gastélum, David Delgado-De la Mora, Adela Rascón-Alcantar, Gerardo Álvarez-Hernández
INTRODUCTION: Rocky Mountain spotted fever is a highly lethal infectious disease, particularly if specific treatment with doxycycline is given belatedly. OBJECTIVE: To describe the clinical profile of fatal Rocky Mountain spotted fever cases in hospitalized patients in the state of Sonora, México. MATERIALS AND METHODS: We conducted a cross-sectional study on a series of 47 deaths caused by Rickettsia rickettsii from 2013 to 2016. The diagnosis of Rocky Mountain spotted fever was confirmed in a single blood sample by polymerase chain reaction (PCR) or by a four-fold increase in immunoglobulin G measured in paired samples analyzed by indirect immunofluorescence...
March 15, 2018: Biomédica: Revista del Instituto Nacional de Salud
https://www.readbyqxmd.com/read/29667134/two-phase-i-pharmacokinetic-and-pharmacodynamic-studies-of-dfp-10917-a-novel-nucleoside-analog-with-14-day-and-7-day-continuous-infusion-schedules
#20
Kamalesh Sankhala, Chris H Takimoto, Alain C Mita, Henry Xiong, Jordi Rodón, Amir Mehrvarz Sarshekeh, K Burns, Kenzo Iizuka, Scott Kopetz
Purpose DFP-10917 is a novel deoxycytidine analog with a unique mechanism of action. Brief exposure to high concentrations of DFP-10917 inhibits DNA polymerase resulting in S-phase arrest, while prolonged exposure to DFP-10917 at low concentration causes DNA fragmentation, G2/M-phase arrest, and apoptosis. DFP-10917 demonstrated activity in tumor xenografts resistant to other deoxycytidine analogs. Experimental design Two phase I studies assessed the safety, pharmacokinetic, pharmacodynamic and preliminary efficacy of DFP-10917...
April 18, 2018: Investigational New Drugs
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