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https://www.readbyqxmd.com/read/29057546/clinical-features-and-biological-implications-of-different-u2af1-mutation-types-in-myelodysplastic-syndromes
#1
Bing Li, Jinqin Liu, Yujiao Jia, Jingya Wang, Zefeng Xu, Tiejun Qin, Zhongxun Shi, Zhen Song, Shuailing Peng, Huijun Huang, Liwei Fang, Hongli Zhang, Lijuan Pan, Naibo Hu, Shiqiang Qu, Yue Zhang, Jian Wu, Na Liu, Kun Ru, Gang Huang, Zhijian Xiao
U2AF1 mutations (U2AF1MT) occur commonly in myelodysplastic syndromes (MDS) without ring sideroblasts. The aim of this study was to investigate the clinical and biological implications of different U2AF1 mutation types in MDS. We performed targeted gene sequencing in a cohort of 511 MDS patients. Eighty-six patients (17%) were found to have U2AF1MT, which occurred more common in younger patients (P=0.001) and represented ancestral lesions in a substantial proportion (71%) of cases. ASXL1MT and isolated +8 were significantly enriched in U2AF1MT-positive cases, whereas TP53MT, SF3B1MT and complex karyotypes were inversely associated with U2AF1MT...
October 23, 2017: Genes, Chromosomes & Cancer
https://www.readbyqxmd.com/read/29057084/changes-in-the-haematological-parameters-of-hiv-1-infected-children-at-6-and-12-months-of-antiretroviral-therapy-in-a-large-clinic-cohort-north-central-nigeria
#2
EDITORIAL
Augustine O Ebonyi, Stephen Oguche, Martha O Ochoga, Oche O Agbaji, Joseph A Anejo-Okopi, Isaac O Abah, Prosper I Okonkwo, John A Idoko
BACKGROUND: Prior to commencing antiretroviral therapy (ART), haematological abnormalities are a common occurrence in individuals diagnosed with human immunodeficiency virus (HIV). In the course of receiving ART, these abnormalities usually improve. We determined the prevalence of haematological abnormalities in children diagnosed with HIV-1 and the changes in haematological parameters that occur after 6 and 12 months of being on ART. METHODS: A cross-sectional study of HIV-1 infected children aged 2 months to 15 years, between July 2005 and March 2013, at the paediatric HIV clinic of the Jos University Teaching Hospital, Jos...
October 1, 2017: Journal of Virus Eradication
https://www.readbyqxmd.com/read/29056075/an-updated-review-of-the-jak1-2-inhibitor-ruxolitininb-in-the-philadelphia-negative-myeloproliferative-neoplasms
#3
Natalia Curto-Garcia, Claire N Harrison
Ruxolitinib (Rux), a JAK1/2 inhibitor, has been approved for patients with myelofibrosis and in polycythemia vera with inadequate response/intolerance to hydroxycarbamide. Studies have demonstrated that Rux improves disease-related symptoms and splenomegaly. A late emerging observation from two Phase III trials was that Rux was associated with survival advantage in comparison with placebo or other available therapies in myelofibrosis. Important data suggest that for polycythemia vera Rux improved control of blood counts...
October 23, 2017: Future Oncology
https://www.readbyqxmd.com/read/29054297/hemolytic-uremic-syndrome-due-to-shiga-toxin-producing-escherichia-coli-infection
#4
REVIEW
M Bruyand, P Mariani-Kurkdjian, M Gouali, H de Valk, L A King, S Le Hello, S Bonacorsi, C Loirat
The leading cause of hemolytic uremic syndrome (HUS) in children is Shiga toxin-producing Escherichia coli (STEC) infection, which has a major outbreak potential. Since the early 2010s, STEC epidemiology is characterized by a decline of the historically predominant O157 serogroup and the emergence of non-O157 STEC, especially O26 and O80 in France. STEC contamination occurs through the ingestion of contaminated food or water, person-to-person transmission, or contact with ruminants or their contaminated environment...
October 17, 2017: Médecine et Maladies Infectieuses
https://www.readbyqxmd.com/read/29051993/treatment-related-sinusoidal-obstruction-syndrome-in-children-with-de-novo-acute-lymphoblastic-leukemia-during-intensification
#5
Casey L McAtee, Netta Schneller, Julienne Brackett, M Brooke Bernhardt, Eric S Schafer
PURPOSE: Sinusoidal obstruction syndrome (SOS), also known as veno-occlusive disease, has been described following treatment of acute lymphoblastic leukemia (ALL) with the anti-metabolite 6-thioguanine (6-TG). Previous studies incorporating daily 6-TG into maintenance chemotherapy demonstrated a high incidence of SOS, typically presenting after prolonged exposures to 6-TG. 6-TG continues to be used as a single, 14-day burst during intensification; however, SOS associated with brief courses of 6-TG is poorly described...
October 19, 2017: Cancer Chemotherapy and Pharmacology
https://www.readbyqxmd.com/read/29051891/scleroderma-renal-crisis-in-mixed-connective-tissue-disease-with-full-renal-recovery-within-3-months-a-case-report-with-expanding-treatment-modalities-to-treat-each-clinical-sign-as-an-independent-entity
#6
Jordana Cheta, Suresh Rijhwani, Harlan Rust
Mixed connective tissue disease (MCTD) is a rheumatologic overlap syndrome that can present with symptoms of systemic lupus erythematous, scleroderma, and polymyositis. A severe but rare complication that can occur in MCTD is scleroderma renal crisis. With multiple poor prognostic indicators, the renal outcome is usually poor. The clinical and histological picture is one of a thrombotic microangiopathy. Clinical suspicion has to be high for additional thrombotic or autoimmune processes coexisting due to associated morbidity...
October 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/29050802/episode-of-kasabach-merritt-phenomenon-following-japanese-encephalitis-vaccination-case-report
#7
Xiaojie Yue, Xiong Zhao, Yefeng Dai, Qiang Shu
Kasabach-Merritt phenomenon (KMP) is a rare potentially life-threatening consumptive coagulopathy characterized by thrombocytopenia and hypofibrinogenemia occurring associated with the vascular tumors kaposiform hemangioendothelioma (KHE) and tufted angioma (TA). A 10-month old male infant, diagnosed with KHE on his left leg, underwent a rapid increase of the lesion and severe thrombocytopenia, one day after the first dose of inactivated Japanese encephalitis (JE) vaccination. The episode of KMP was treated successfully by steroid...
October 16, 2017: Vaccine
https://www.readbyqxmd.com/read/29050793/anaplasmosis-in-pediatric-patients-case-report-and-review
#8
Vaka K Sigurjonsdottir, Henry M Feder, Gary P Wormser
Human granulocytic anaplasmosis (HGA) is a tick-borne infection, characterized as an acute and sometimes severe febrile illness which may be associated with leukopenia and thrombocytopenia. Most case reports of HGA have been in adults, with only 8 case reports of HGA in children. We add a ninth case of HGA, which occurred in a 5-year-old. The paucity of pediatric HGA case reports maybe because publication bias, or HGA in children is a mild illness and children with HGA are less likely than adults to seek medical care, or the diagnosis of HGA requires a blood draw and adults (versus children) are more likely to get diagnostic testing...
November 2017: Diagnostic Microbiology and Infectious Disease
https://www.readbyqxmd.com/read/29050748/pediatric-kikuchi-fujimoto-disease-a-clinicopathologic-study-and-the-therapeutic-effects-of-hydroxychloroquine
#9
Yung-Chih Lin, Hsiu-Hui Huang, Bao-Ren Nong, Po-Yen Liu, Ying-Yao Chen, Yung-Feng Huang, Yee-Hsuan Chiou, Herng-Sheng Lee
BACKGROUND: To investigate the clinical features of Kikuchi-Fujimoto disease (KFD) in children, and place an emphasis on the therapeutic effects of hydroxychloroquine as monotherapy. METHODS: We retrospectively reviewed the medical records of all children diagnosed with KFD during the period January 1992 to September 2016 at a tertiary medical center in Taiwan. RESULTS: 40 patients were histopathologically confirmed as KFD, and the mean age of the patients was 13...
September 29, 2017: Journal of Microbiology, Immunology, and Infection, Wei Mian Yu Gan Ran za Zhi
https://www.readbyqxmd.com/read/29050535/antiphospholipid-antibodies-and-non-thrombotic-manifestations-of-systemic-lupus-erythematosus
#10
U İlgen, M E Yayla, A Ateş, İ E Okatan, E U Yurteri, M Torgutalp, A B D Keleşoğlu, T M Turgay, G Kınıklı
Objectives The aim of this study was to investigate the association between antiphospholipid antibodies and non-thrombotic and non-gestational manifestations of systemic lupus erythematosus. Methods Systemic lupus erythematosus patients with persistently positive antiphospholipid antibodies or lupus anticoagulant were identified and grouped as systemic lupus erythematosus with antiphospholipid syndrome (SLE-APS), systemic lupus erythematosus with positive antiphospholipid antibodies/lupus anticoagulant without antiphospholipid syndrome (SLE-aPL), and systemic lupus erythematosus with negative aPLs (SLE-No aPL)...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/29050347/efficacy-and-safety-of-dose-dense-chemotherapy-in-urothelial-carcinoma
#11
Chenjing Zhu, Jiaming Liu, Jing Zhang, Qingfang Li, Qisi Lian, Jing Xu, Xuelei Ma
We conducted a meta-analysis to assess the efficacy and safety of dose-dense chemotherapy in the treatment of patients with urothelial carcinoma. A systematic search was conducted in PubMed, Medline, Embase, Web of Science and Cochrane Collaboration's Central register of controlled trials (CENTRAL) for relevant articles. Data was obtained from 10 trials with a total of 1093 patients. The pooled pathologic complete response (pCR) was 27.8% in the ten studies with a full cohort of 684 patients who received dose-dense methotrexate, vinblastine, adriamycin and cisplatin (dd-MVAC)...
September 19, 2017: Oncotarget
https://www.readbyqxmd.com/read/29050117/-pediatric-idiopathic-hypereosinophilic-syndrome-with-pulmonary-embolism-a-case-report-and-review-of-literature
#12
N Dong, X Y Dong
Objective: To explore clinical features of idiopathic hypereosinophilic syndrome combined with pulmonary embolism. Method: A retrospective analysis of a patient with idiopathic hypereosinophilic syndrome and pulmonary embolism diagnosed and treated in the Respiratory Department of Shanghai Children's Hospital in September 2016 was performed. A literature search was performed with"Eosinophils increased, thrombosis"as the Chinese keywords in Wanfang database and"idiopathic hypereosinophilic syndrome, deep vein thrombosis"as the English key words in PubMed database...
October 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29049666/bivalirudin-anticoagulation-for-left-ventricular-assist-device-implantation-on-an-extracorporeal-life-support-system-in-patients-with-heparin-induced-thrombocytopenia-antibodies
#13
Edis Ljajikj, Armin Zittermann, Michiel Morshuis, Jochen Börgermann, Maria Ruiz-Cano, Michael Schoenbrodt, Jan Gummert, Andreas Koster
OBJECTIVES: Heparin-induced thrombocytopenia (HIT) requires alternative anticoagulation strategies. We investigated outcomes in patients with HIT antibodies undergoing low-dose bivalirudin anticoagulation during left ventricular assist device implantation on an extracorporeal life support system (ECLS) and compared the results with non-HIT patients treated with heparin and receiving left ventricular assist device implantation with ECLS support. METHODS: The institutional ventricular assist device database was searched for the period from March 2012 to March 2016...
July 28, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29049237/effect-and-safety-of-cytokine-induced-killer-cik-cell-immunotherapy-in-patients-with-breast-cancer-a-meta-analysis
#14
Jianxin Hu, Junfeng Hu, Xiandong Liu, Chao Hu, Ming Li, Wei Han
BACKGROUND: Breast cancer (BC) is considered a systemic disease with a primarily locoregional component. The accumulation of basic researches and clinical studies related to cytokine-induced killer (CIK) cells has confirmed their safety and feasibility in treating BC. By searching the PubMed, Embase, CNKI, and Wanfang databases, we conducted a meta-analysis to assess the efficacy and safety of DC/CIK plus chemotherapy regimen (Exp) compared with chemotherapy (Con) alone regimen for breast carcinoma...
October 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29049216/fondaparinux-is-effective-for-acute-portal-vein-thrombosis-in-decompensated-cirrhotic-patients
#15
Zhi-Hao Zhang, Jing-Wen Zhang, Ping He, Yan Zhou, Chang-Yu Sun
Portal vein thrombosis (PVT) is a rare but serious complication in the decompensated stage of cirrhosis, and recurrent upper gastrointestinal bleeding and refractory ascites can occur in such patients. In decompensated cirrhotic patients, the application of conventional anticoagulant therapy is limited due to severe coagulation disorders, thrombocytopenia, and history of gastrointestinal bleeding.In this study, we sought to investigate the effect of fondaparinux on acute PVT in decompensated cirrhotic patients...
October 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29049199/paclitaxel-as-third-line-chemotherapy-for-small-cell-lung-cancer-failing-both-etoposide-and-camptothecin-based-chemotherapy
#16
Se Hyun Kim, Mi-Jung Kim, Yu Jung Kim, Hyun Chang, Jin Won Kim, Jeong-Ok Lee, Keun-Wook Lee, Jee Hyun Kim, Soo-Mee Bang, Jong Seok Lee
Paclitaxel has been shown to have clinical activity in the treatment of small cell lung cancer (SCLC). However, its role as third-line chemotherapy for SCLC after both etoposide- and camptothecin-based regimens has not been clarified.All patients with refractory SCLC who were treated with paclitaxel-based regimen as third-line chemotherapy between 2005 and 2011 in Seoul National University Bundang Hospital were reviewed retrospectively. Forty patients previously treated with both etoposide- and camptothecin-based chemotherapy were included...
October 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29048130/beneficial-effect-of-exogenous-platelet-factor-4-for-detecting-pathogenic-heparin-induced-thrombocytopenia-antibodies
#17
Caroline Vayne, Eve-Anne Guery, Claire Kizlik-Masson, Jérôme Rollin, Anne Bauters, Yves Gruel, Claire Pouplard
The laboratory diagnosis of heparin-induced thrombocytopenia (HIT) is based on an enzyme immunoassay combined with a functional test, and serotonin release assay (SRA) is the gold standard for detecting activating HIT antibodies. However, a recent atypical history of HIT prompted us to evaluate whether addition of platelet factor 4 (PF4) during SRA could improve its ability to detect pathogenic HIT antibodies. Using 5B9, a monoclonal antibody to PF4/H with a human Fc fragment, we first defined the optimal PF4 concentration for detecting low amounts of platelet-activating IgG with SRA...
October 19, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/29046759/copper-deficiency-a-new-triad-anemia-leucopenia-and-myeloneuropathy
#18
Shoaib M Wazir, Ibrahim Ghobrial
Clinical copper deficiency is now more frequently recognized. Hematologically, it can present as anemia (microcytic, normocytic, or macrocytic) and neutropenia. Thrombocytopenia is relatively rare. Neurologically, it can manifest as myelopathy and peripheral neuropathy simulating subacute combined degeneration. Bone marrow findings can mimic myelodysplasia resulting in occasional inappropriate referral for bone marrow transplantation. Other conditions with similar presentations include infections, drug toxicity, autoimmunity, B12 deficiency, folate deficiency, myelodysplastic syndrome, aplastic anemia, and lymphoma with bone marrow involvement...
October 2017: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/29046203/-clinical-effect-of-anti-d-immunoglobulin-in-treatment-of-childhood-immune-thrombocytopenia-a-meta-analysis
#19
Wei Qin, Shao-Ling Huang, Ting-Ting Li
OBJECTIVE: To investigate the clinical effect and safety of anti-D immunoglobulin (anti-D) in the treatment of children with newly diagnosed acute immune thrombocytopenia (ITP) through a Meta analysis. METHODS: PubMed, EMBASE, Cohrane Library, Ovid, CNKI, and Wanfang Data were searched for randomized controlled trials (RCTs) published up to April 2017. Review Manager 5.3 was used for the Meta analysis. RESULTS: Seven RCTs were included. The Meta analysis showed that after 72 hours and 7 days of treatment, the intravenous immunoglobulin (IVIG) group had a significantly higher percentage of children who achieved platelet count >20×10(9)/L than the anti-D group (P<0...
October 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/29046071/clinical-significance-of-the-serotonin-release-assay-and-platelet-count-monitoring-after-cardiac-surgery
#20
Shinya Motohashi, Takefumi Matsuo, Hidenori Inoue, Makoto Kaneko, Shunya Shindo
Heparin-induced thrombocytopenia (HIT) is one of the serious complications in patients who undergo cardiac surgery. However, there remains a major problem in diagnosing HIT because the current immunological assays for detection of HIT antibody have limitations. Furthermore, the clinical course of thrombocytopenia in this surgery makes it increasingly difficult to diagnose HIT. We investigated the relationship between platelet count and HIT antibody in 59 patients who underwent cardiac surgery using cardiopulmonary bypass (CPB)...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
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