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Hematology (Amsterdam, Netherlands)

Q A Hill, L C Harrison, A D Padmakumar, R G Owen, K R Prasad, G F Lucas, P Tachtatzis
OBJECTIVE AND IMPORTANCE: Transplantation-mediated alloimmune thrombocytopenia (TMAT) occurs when leukocytes transferred in a donor organ from a patient with immune thrombocytopenia (ITP), mount a response against recipient platelets. We present the first fatal case of TMAT following liver transplantation and review its aetiology and treatment. CLINICAL PRESENTATION: The liver donor had ITP and died from an intracranial haemorrhage. The recipient platelet count fell to 2 × 10(9)/l on post-operative day 2...
October 21, 2016: Hematology (Amsterdam, Netherlands)
Luba Nalysnyk, Philip Rotella, Jason C Simeone, Alaa Hamed, Neal Weinreb
OBJECTIVES: The objectives of this research were: (1) to heighten awareness of Gaucher disease (GD), a rare lysosomal storage disorder with highly heterogeneous patterns of organ involvement and disease severity, to clinicians most likely to encounter these patients, and; (2) to summarize the published evidence on GD epidemiology which is essential to accurately depict the total societal burden of this rare worldwide disorder. METHODS: A comprehensive literature review was undertaken to summarize the published evidence on the epidemiology of GD...
October 20, 2016: Hematology (Amsterdam, Netherlands)
Maryam Akbari, Mahmood Moosazadeh, Reza Tabrizi, Seyed Reza Khatibi, Mahmoud Khodadost, Seyed Taghi Heydari, Ahmad Naghibzadeh Tahami, Kamran B Lankarani
OBJECTIVES: Iron deficiency anemia (IDA) is a major health issue in those aged less than 18 years old with high impact on their development. There are several reports from Iran with variable results. Systematic review and met analysis of these data would reveal a more realistic view of the prevalence of iron deficiency (ID). METHODS: We conducted a systematic search of national and international databases from December 1990 to 31 January 2016 for population-based studies providing estimates on the prevalence of IDA in Iran...
October 14, 2016: Hematology (Amsterdam, Netherlands)
Wenle Zhao, Vance W Berger
No abstract text is available yet for this article.
October 14, 2016: Hematology (Amsterdam, Netherlands)
Gang Deng, Shifang Yu, Qiming Li, Yunlei He, Wei Liang, Lu Yu, Deyi Xu, Tao Sun, Ri Zhang, Qiang Li
OBJECTIVES: Immune thrombocytopenia (ITP) is an acquired and heterogeneous autoimmune-mediated hematological disease typically characterized by a low platelet count. Emerging evidence over the past several years suggests that platelet biogenesis and ageing are regulated, at least in part, by apoptotic mechanisms. However, the association between decreased platelets and apoptosis in ITP patients is poorly understood. To better understand the role of platelet apoptosis in ITP pathophysiology, we investigated apoptotic markers in platelets acquired from 40 chronic ITP patients...
October 13, 2016: Hematology (Amsterdam, Netherlands)
Wangqiang Hu, Xiaoxia Wang, Rongrong Yang, Laixi Bi, Yaosheng Xie, Zhuo Zhang, Hong Lu, Lianfeng Wu
OBJECTIVE: To gain further insights into the predisposing risk factors for central nervous system (CNS) involvement in patients with acute lymphocytic leukemia (ALL), the impact of CD56 expression in these patients was investigated. METHODS: We reviewed the clinical features of CD56 expression in 588 consecutive ALL patients treated with systemic chemotherapy regimens between 2000 and 2014. The categorical data from CD56(+) ALL patients were compared with those from CD56(-) ALL patients...
October 13, 2016: Hematology (Amsterdam, Netherlands)
Leylagul Kaynar, Koray Demir, Esra Ermiş Turak, Çiğdem Pala Öztürk, Gökmen Zararsız, Zeynep Burçin Gönen, Selma Gökahmetoğlu, Serdar Şıvgın, Bülent Eser, Yavuz Köker, Musa Solmaz, Ali Ünal, Mustafa Çetin
INTRODUCTION: The use of αβ+ T-cell-depleted grafts is a novel approach to prevent graft failure, graft-versus-host disease (GVHD), and non-relapse mortality (NRM) in patients undergoing haploidentical hematopoietic stem cell transplantation. PATIENT AND METHOD: Thirty-four patients with acute leukemia and lacking a match donor were treated with αβ T-cell-depleted allografts from haploidentical family donors. A total of 24 patients had acute myeloid leukemia (AML) and 10 had acute lymphoblastic leukemia...
October 10, 2016: Hematology (Amsterdam, Netherlands)
Ji Won In, Nuri Lee, Eun Youn Roh, Sue Shin, Kyoung Un Park, Eun Young Song
OBJECTIVES: Aplastic anemia (AA) is characterized by pancytopenia and bone marrow failure, and most acquired AA is an immune-mediated disorder. Regulatory T cells (Tregs) suppressing autoreactive T cells were decreased in AA patients. FoxP3 is a major regulator for the development and function of Tregs. Polymorphism in FoxP3 was shown to be associated with various autoimmune diseases, however, has not yet been studied in AA. In this study, we examined the association between FoxP3 polymorphisms and AA in Korean patients...
October 5, 2016: Hematology (Amsterdam, Netherlands)
Jing Chen, Yang Zhou, Yaqi Gao, Weijie Cao, Hui Sun, Yanfang Liu, Chong Wang
OBJECTIVE: Hereditary spherocytosis (HS) is a hemolytic disorder characterized by the presence of spherical-shaped red blood cells on the peripheral blood smear. Non-dominant HS cases are due to de novo mutations of the type associated with dominant inheritance or recessive genes. This study is aimed to identify HS-related biological mechanisms and predicting HS candidate genes. METHODS: We searched the known HS-related genes from the public databases. By analyzing the gene ontology (GO) and biological pathway of these genes, we extracted the optimal features to encode HS genes...
October 3, 2016: Hematology (Amsterdam, Netherlands)
Parin Kamseng, Satariya Trakulsrichai, Objoon Trachoo, Walaiporn Yimniam, Bhakbhoom Panthan, Paisan Jittorntam, Pimjai Niparuck, Pitsucha Sanguanwit, Winai Wananukul, Sumalee Jindadamrongwech
OBJECTIVE: To investigate the cause(s) of a Thai male proband presenting low oxygen saturation by pulse oximetry (SpO2) and severe anemia. METHODS: As Hb variant was suspected, Hb typing was determined by high-performance liquid chromatography and capillary electrophoresis, and subsequently Hb variant was identified by DNA sequencing. Complete blood counts were performed using automated blood cell counter and oxygen saturation was measured by pulse oximetry. RESULTS: Proband was compound heterozygous for Hb Louisville [β42(CD1)Phe→Leu] and Hb La Desirade [β129(H7)Ala→Val]...
September 27, 2016: Hematology (Amsterdam, Netherlands)
María Perera, Teresa Garrido
OBJECTIVES: Classically, immune thrombocytopenia (ITP) was thought to be caused by the destruction and insufficient production of platelets, as mediated by autoantibodies. More recently other immune mechanisms that contribute to the disease have been discovered. This review attempts to address the main unresolved questions in ITP. METHODS: We review the most current knowledge of the pathophysiology of ITP. Immunological effects of available therapies are also described...
September 27, 2016: Hematology (Amsterdam, Netherlands)
Lama Al-Faris, Monera Al-Rukhayes, Salah Al-Humood
OBJECTIVES: To investigate the pattern of CD55 and CD59 expression on RBCs of SCD patients, and its association with anemia, biochemical parameters of hemolysis, level of erythropoietin, and pro-inflammatory markers. METHODS: Flow cytometric analysis was performed on RBCs from 71 adult SCD patients and 53 healthy controls, using the commercial REDQUANT kit. RESULTS: CD59 deficiency was significantly higher among SCD patients than among healthy controls...
September 25, 2016: Hematology (Amsterdam, Netherlands)
Chunling Wang, Zhengmei He, Yuye Shi, Lijuan Zhang, Yue Chen, Zhi Chen, Liang Yu
OBJECTIVE: This study investigated the efficacy and safety of low-dose lenalidomide combined with dexamethasone in elderly patients with relapsed and refractory multiple myeloma (MM). METHODS: Thirty-two elderly patients with refractory and recurrent MM (median age: 64 years) were treated with low-dose lenalidomide (LD-R) combined with dexamethasone (D). LD-R (10 mg/d) was administered orally for 21 days and D (40 mg/d) was administered twice a day on days 1-4, 9-12, and 17-20...
September 25, 2016: Hematology (Amsterdam, Netherlands)
Omid Reza Zekavat, Seyed Javad Dehghani, Jaber Imanifard, Javad Dehbozorgian, Soheila Zareifar, Sezaneh Haghpanah
OBJECTIVE AND IMPORTANCE: Thalassemia is the most frequently monogenetic disorders around the world that is inherited as a recessive single-gene disease, resulting from mutations in α- or β-globin gene clusters. The aim of this report was to present a new insertional mutation in the α1 globin gene which causes transfusion-dependent anemia in α-thalassemic patients. CLINICAL PRESENTATION: Two 5-year-old girls with blood transfusion-dependent α-thalassemia anemia and another girl with moderate α-thalassemia have been presented among patients who have been referred to Hematology and Thalassemia Research Center, Dastgheib Hospital, Shiraz, Iran...
September 25, 2016: Hematology (Amsterdam, Netherlands)
M Pamukcuoglu, K Acar, B Celik, N Akyurek, M S Pepeler, G T Sucak
PURPOSE: The aim of the present study was to examine the relationship between peripheral CD34(+) and bone marrow CD34(+) levels and the clinicopathologic characteristics and laboratory parameters of myeloproliferative disease (MPD) patients. PATIENTS AND METHODS: A total of 103 MPD patients were enrolled in this study. We examined the relationship between bone marrow CD34(+) and peripheral CD34(+) levels and the patients' clinicopathologic and laboratory parameters...
September 22, 2016: Hematology (Amsterdam, Netherlands)
Hamid Amoozgar, Samaneh Zeighami, Sezaneh Haghpanah, Mehran Karimi
BACKGROUND: The goal of this study was to compare heart function and arrhythmia in clinically asymptomatic patients with beta thalassemia intermedia and beta thalassemia major. METHOD: In this cross-sectional study, 60 patients with beta thalassemia major and 60 patients with beta thalassemia intermedia who had clinically no symptoms of arrhythmia and clinically normal heart function were evaluated using 24-hour ambulatory electrocardiogram monitoring and echocardiography...
September 20, 2016: Hematology (Amsterdam, Netherlands)
Salah Aref, Mohamed Sabry El-Ghonemy, Sherin Abd El-Aziz, Tarek Abouzeid, Mona Talaab, Amr El-Sabbagh
OBJECTIVES: Based on the concept of immune dysregulation in immune thrombocytopenic purpura (ITP) and that Interleukin-18 (IL-18) is an inflammatory cytokine that plays an important role in autoimmune disease by inducing interferon-γ secretion; this study aimed to assess a possible association between the IL-18 promoter polymorphisms (-607 C/A site) and genetic susceptibility to ITP and the impact of the immunoglobulins (Igs) concentrations level on disease severity and response to therapy...
September 6, 2016: Hematology (Amsterdam, Netherlands)
César Homero Gutierrez-Aguirre, José Alberto García-Lozano, Oscar Rubén Treviño-Montemayor, José Luis Iglesias-Benavides, Olga Graciela Cantú-Rodríguez, Oscar González-Llano, Andrés Gómez-De León, Rosario Salazar-Riojas, Consuelo Mancias-Guerra, José Carlos Jaime-Pérez, Abel Guzman-López, David Gómez-Almaguer
OBJECTIVE: To compare serum ferritin (SF) concentrations and other hematological parameters between patients with preeclampsia (PE) and normal pregnant women of the same gestational period who received supplemental iron during pregnancy. METHODS: Prospective, comparative, observational pilot study that included 31 women with PE and 30 healthy pregnant women, at 20 weeks' of gestation. Ferritin, iron and complete blood cell count were compared between groups. RESULTS: In comparison with controls, preeclamptic patients had a higher weight, body mass index, and arterial pressure...
August 24, 2016: Hematology (Amsterdam, Netherlands)
Shucheng Gu, Yanjun Xv, Chengming Fei, Chao Xiao, Juan Guo, Youshan Zhao, Feng Xv, Xiao Li, Chunkang Chang
OBJECTIVES: In order to gain an insight into labile plasma iron (LPI) in iron metabolism microenvironment in MDS. METHODS: We performed ELISA, quantitative real-time polymerase chain reaction, flow cytometry, MRI T2* assays to test LPI, iron biochemical parameters, and liver iron concentration (LIC) among 22 MDS patients. RESULTS: LPI has a statistical difference (P < 0.001 by analysis of variance (ANOVA)), which decreased gradually, among three groups, while no difference was found in adjusted serum ferritin (ASF) (P = 0...
August 11, 2016: Hematology (Amsterdam, Netherlands)
Adrian Newland
No abstract text is available yet for this article.
October 2016: Hematology (Amsterdam, Netherlands)
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