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Hematology (Amsterdam, Netherlands)

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https://www.readbyqxmd.com/read/28795658/double-vs-single-cord-blood-transplantation-in-adolescent-and-adult-hematological-malignancies-with-heavier-body-weight-%C3%A2-50%C3%A2-kg
#1
Chang-Cheng Zheng, Xiao-Yu Zhu, Bao-Lin Tang, Xu-Han Zhang, Lei Zhang, Liang-Quan Geng, Hui-Lan Liu, Zi-Min Sun
BACKGROUND: Double-unit cord blood transplantation (CBT) can be used to overcome the limitation of single-unit CBT with low cell content for adults and larger adolescents. However, whether double-unit CBT is superior to single-unit CBT remains controversial. METHODS: We reviewed the medical records of 228 consecutive hematological malignancies who received CBT between November 2005 and December 2013. Ninety-seven eligible patients met the criteria (age ≥14 years and body weight ≥50kg) and were enrolled in this study...
August 10, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28768465/interaction-between-hb-e-and-hb-yala-hbb-c-129delt-a-novel-frameshift-beta-globin-gene-mutation-resulting-in-hemoglobin-e-%C3%AE-0-thalassemia
#2
Supachai Ekwattanakit, Suchada Riolueang, Vip Viprakasit
OBJECTIVES: There are more than 200 known mutations found in patients with β-thalassemia, a possibility to identify an unknown or novel mutation becomes less possible. Here, we report a novel mutation in a patient from Thailand who presented with chronic hemolytic anemia. METHODS: A comprehensive hematology and DNA analysis was applied in the index patient and her mother. RESULTS: Hematological and hemoglobin analyses were consistent with the clinical diagnosis of Hb E/β(0)-thalassemia...
August 3, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28766464/stigma-and-illness-uncertainty-adding-to-the-burden-of-sickle-cell-disease
#3
Alphanso Blake, Vikram Asnani, Robin R Leger, June Harris, Victoria Odesina, Jennifer Knight-Madden, Linda Wagner, Monika Rani Asnani
BACKGROUND: Persons with sickle cell disease (SCD) experience multiple medical and physical complications; the disease also has numerous effects on their social and emotional well-being. We hypothesized that adults with SCD in Jamaica experience moderate levels of stigma and illness uncertainty and that these experiences may be associated with socio-demographic factors, such as gender, educational status and economic status. METHODS: We surveyed 101 adults with SCD (54...
August 2, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28762299/congenital-prothrombin-defects-they-are-not-only-associated-with-bleeding-but-also-with-thrombosis-a-new-classification-is-needed
#4
Antonio Girolami, Silvia Ferrari, Elisabetta Cosi, Bruno Girolami, Anna Maria Lombardi
OBJECTIVE: Congenital prothrombin deficiency is one of the rarest clotting disorders. It is commonly subdivided in Type I defects or cases of 'true' prothrombin deficiency characterized by a concomitant decrease in FII activity and antigen and in Type II or dysprothrombinemias, in which FII activity is low but FII antigen is normal or near normal. A bleeding tendency, often a severe one, is the hallmark of the two-defects even though the bleeding is usually less severe in the Type 2 defects or dysprothrombinemias...
August 1, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28759343/epidemiologic-study-of-major-complications-in-adolescent-and-adult-patients-with-thalassemia-in-northeastern-thailand-the-e-saan-study-phase-i
#5
Nattiya Teawtrakul, Arunee Jetsrisuparb, Saranya Pongudom, Chittima Sirijerachai, Kanchana Chansung, Chinadol Wanitpongpun, Supan Fucharoen
INTRODUCTION: Thalassemia-related complications are one of the main factors that increase morbidity and mortality in aging patients with thalassemia. This study was aimed to report the prevalence and clinical risk factors for the complications in thalassemia. METHODS: A multi-center prospective cohort study was conducted in patients with thalassemia aged ≥10 years old. Thalassemia-related complications were heart failure, pulmonary hypertension, extramedullary hematopoiesis, endocrine disorders, infections, thrombosis and leg ulcers...
July 31, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28728506/microsatellite-instability-and-promoter-hypermethylation-of-dna-repair-genes-in-hematologic-malignancies-a-forthcoming-direction-toward-diagnostics
#6
Priyanjali Bhattacharya, Trupti N Patel
OBJECTIVE: The objective of our review is to highlight the significance of microsatellite hypervariation in diagnostics of hematologic malignancies. METHODS: For the past few decades, extensive experiments in cancer research have explored all the possible pathways and a number of deleterious mutations that either make the tumor suppressor genes (TSGs) dysfunctional or cause the proto-oncogenes to behave abnormally by changing the cellular phenotype hence rendering disease...
July 20, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28718348/thalidomide-has-a-significant-effect-in-patients-with-thalassemia-intermedia
#7
YunShuan Li, Quan Ren, Yali Zhou, Pingping Li, Wanhua Lin, Xiaolin Yin
OBJECTIVE: To investigate the effect of thalidomide in patients with thalassemia intermedia. METHODS: We observed the effect of thalidomide in seven patients with thalassemia intermedia requiring blood transfusion. Four of the patients were transfusion-independent, and three patients were transfusion-dependent. RESULTS: For the four transfusion-independent patients, hemoglobin concentration increased significantly (≥2 g/dl) in three and moderately (1-2 g/dl) in one...
July 18, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28675126/characteristics-of-bone-marrow-cell-dysplasia-and-its-effectiveness-in-diagnosing-myelodysplastic-syndrome
#8
Chujia Liang, Junxun Li, Jing Cheng, Shaoqian Chen, Zhuangjian Ye, Fan Zhang, Zhe Wang, Fang Wang, Cheng Peng, Juan Ouyang
BACKGROUND: Although dysplasia plays an important role in the diagnosis of myelodysplasia syndrome (MDS), its morphologic variety and irregularity result in difficulties in its clinical application. METHODS: Bone marrow smears from cases with MDS and non-clonal disease were collected and performed microscopy analysis. We respectively recorded the percentage of specific dysplastic cells (PSDC) and incidence of specific dysplasia (ISD) of each dysplastic type in three hematopoietic cell lineages for the comprehensive analysis of diagnostic efficacy to MDS...
July 4, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28651453/pretransplant-serum-ft3-levels-in-recipients-predict-early-non-relapse-mortality-after-myeloablative-allogeneic-haematopoietic-cell-transplantation-from-matched-sibling-donors
#9
Huijuan Li, Jia Chen, Bimin Shi, Xingbo Chen, Depei Wu, Ying Wang
OBJECTIVES: Although decreased thyroid function is negatively correlated with clinical outcomes in critically ill patients, its role in allogeneic haematopoietic cell transplantation (allo-HCT) has not been sufficiently described. METHODS: The associations between pre-conditioning thyroid hormone concentrations and transplant-related complications in 474 adult patients with haematologic malignancies who underwent myeloablative allo-HCT were assessed. RESULTS: A receiver-operating characteristic curve showed that the baseline serum-free triiodothyronine 3 (FT3) level had an excellent predictive value for non-relapse mortality (NRM) within 100 days in sibling HCT with an area under the curve of 0...
June 26, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28621205/neurocognitive-dysfunction-in-children-with-%C3%AE-thalassemia-major-psychometric-neurophysiologic-and-radiologic-evaluation
#10
M S Elalfy, R H Aly, H Azzam, K Aboelftouh, R H Shatla, M Tarif, M Abdatty, R M Elsayed
OBJECTIVE: To evaluate the impact of iron chelating drugs and serum ferritin on the neurocognitive functions of patients with β thalassemia major (β-TM), using psychometric, neurophysiologic and radiologic tests. METHODS: Eighty children with β-TM were enrolled into the study and were compared to 40 healthy controls. All participants were evaluated by measuring serum ferritin, neurocognitive assessment by Benton Visual Retention Test, Wechsler Intelligence Scale for Children, Wisconsin Card Sort Test, P300 and magnetic resonance spectroscopy (MRS)...
June 16, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28589785/revisiting-beta-thalassemia-intermedia-past-present-and-future-prospects
#11
Naouel Ben Salah, Rayan Bou-Fakhredin, Fethi Mellouli, Ali T Taher
BACKGROUND: The spectrum of thalassemias is wide ranging from thalassemia minor, which consists of mild hypochromic microcytic anemia without obvious clinical manifestations, to thalassemia major (TM), which is characterized by severe anemia since the first years of life and is transfusion dependent. Thalassemia intermedia (TI) describes those patients with mild or moderate anemia. OBJECTIVE: To describe the genetic features and major clinical complications of TI, and the therapeutic approaches available in the management of this disease...
June 7, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28583051/monoclonal-b-cell-lymphocytosis-in-blood-donors-in-turkey
#12
Münci Yağcı, Zeynep Arzu Yegin, İdil Yenicesu, Elif Suyanı, Bahar Uncu Ulu, Kamil İnci, Zeynep Çetin, Zeynep Yılmaz, Nevruz Kurşunoğlu, Zübeyde Nur Özkurt
OBJECTIVES: Monoclonal B-cell lymphocytosis (MBL) is a precursor state of chronic lymphocytic leukemia (CLL) with peripheral lymphocytosis below 5 × 10(9)/l. The diagnostic criteria exclude the presence of lymphadenopathy, organomegaly, infections, autoimmune diseases or any sign of a lymphoproliferative disorder. This prospective study was designed in order to evaluate the frequency of MBL in blood donors in Turkey. METHODS: The diagnosis of MBL was identified by flow cytometry method based on the International Familial CLL Consortium Report...
June 5, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28581364/positron-emission-tomography-computed-tomography-features-of-monomorphic-epitheliotropic-intestinal-t-cell-lymphoma
#13
Thomas S Y Chan, Elaine Lee, Pek-Lan Khong, Eric W C Tse, Yok-Lam Kwong
OBJECTIVE: To define the positron emission tomography/computed tomography (PET/CT) features of monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), a rare malignancy in European and North American populations and the most common form of primary intestinal T-cell lymphoma in Asian populations. METHODS: (18)F-fluorodeoxyglucose (FDG) PET/CT findings of a cohort of MEITL patients were retrospectively analyzed. RESULTS: Eight men and four women with MEITL investigated by PET/CT at diagnosis and relapse were retrospectively analyzed...
June 5, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28581352/interferon-%C3%AE-mediates-the-immunosuppression-of-bone-marrow-mesenchymal-stem-cells-on-t-lymphocytes-in-vitro
#14
Chen Liang, Erlie Jiang, Jianfeng Yao, Mei Wang, Shulian Chen, Zheng Zhou, Weihua Zhai, Qiaoling Ma, Sizhou Feng, Mingzhe Han
OBJECTIVES: In the present study, we first confirmed the suppressive function of MSCs in allogeneic T cell proliferation and then examined the underlying mechanisms for MSCs' immunomodulation and the role of the pro-inflammatory cytokine interferon (IFN)-γ. METHODS: Human MSCs were cultured in the presence or absence of IFN-γ. The expression level of prostaglandin E2 (PGE2), hepatocyte growth factor (HGF), transforming growth factor (TGF)-β1 and indoleamine 2,3-dioxygenase (IDO) by MSCs were measured...
June 5, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28580844/relapse-of-childhood-acute-lymphoblastic-leukemia-and-outcomes-at-a-reference-center-in-latin-america-organomegaly-at-diagnosis-is-a-significant-clinical-predictor
#15
José Carlos Jaime-Pérez, Mónica Andrea Pinzón-Uresti, Raúl Alberto Jiménez-Castillo, Julia Esther Colunga-Pedraza, Óscar González-Llano, David Gómez-Almaguer
OBJECTIVE: Relapse is the major cause of treatment failure in acute lymphoblastic leukemia (ALL) of childhood; it is more frequent among high-risk patients from low-middle income than from high-income countries. The frequency, sites and outcome of relapsed ALL in children of northeast Mexico over a decade was documented. METHODS: A retrospective analysis of 246 children belonging to a low-income group <16 years with de novo ALL during 2004-2015 was performed...
June 3, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28580841/safety-and-efficacy-of-ofatumumab-in-chronic-lymphocytic-leukemia-a-systematic-review-and-meta-analysis
#16
Yougen Wu, Yang Wang, Yuting Gu, Ju Xia, Xiaoyang Kong, Qingqing Qian, Yang Hong
OBJECTIVES: Increasing numbers of clinical studies have been carried out to investigate the therapeutic effect of Ofatumumab for patients with chronic lymphocytic leukemia (CLL) but no studies have yet reported a pooled estimate of the treatment effect. We performed a meta-analysis of evidence from 13 clinical trials to assess effectiveness and safety of Ofatumumab-based therapy in patients with CLL. METHODS: Relevant publications from PubMed, Web of Science, Embase, and ClinicalTrials...
June 3, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28574302/clinical-characteristics-molecular-profile-and-outcomes-of-myeloid-sarcoma-a-single-institution-experience-over-13-years
#17
Varinder Kaur, Arjun Swami, Daisy Alapat, Al Ola Abdallah, Pooja Motwani, Laura F Hutchins, Yogesh Jethava
BACKGROUND: Myeloid sarcoma (MS) is characterized by extramedullary infiltration by immature myeloid cells. Owing to rarity of this disease, the clinical features and overall outcomes are yet to be clarified. OBJECTIVE: To define clinical characteristics, epidemiology, pathologic findings, treatment options and outcomes in MS. METHODS: We conducted a retrospective review of 23 patients diagnosed with MS at our institute over a period of 13 years (2002-2015)...
June 2, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28569613/the-role-of-bone-marrow-microenvironment-in-platelet-production-and-their-implications-for-the-treatment-of-thrombocytopenic-diseases
#18
Jun-Ying Wang, Shuang Ye, Hua Zhong
OBJECTIVES: Impaired platelet production has been found to be an important pathological mechanism of thrombocytopenia in many diseases. Platelet generation is a complex process that mainly occurs in the bone marrow, and thus is closely regulated by the bone marrow microenvironment. This review attempts to summarize the most current knowledge referring the role of bone marrow microenvironment in the regulation of platelet production. METHODS: The effects of multiple microenvironment ingredients in regulating megakaryopoiesis and thrombocytopoiesis have been discussed...
June 1, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28545322/cardiovascular-events-during-carfilzomib-therapy-for-relapsed-myeloma-practical-management-aspects-from-two-case-studies
#19
Andrzej J Jakubowiak, Jeanne M DeCara, Khalid Mezzi
Objectives and importance: Patients with multiple myeloma (MM) have an increased risk of cardiovascular comorbidities due to disease burden and treatment-related risk factors. Proteasome inhibitors, including bortezomib and carfilzomib, are effective and generally well tolerated anti-MM agents. However, cardiovascular-related toxicities have been reported with this class of agents, the mechanisms of which are not fully understood. We discuss the practical management of cardiovascular events during carfilzomib therapy for relapsed MM...
May 25, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28532344/pre-transplantation-thymic-function-is-associated-with-the-risk-of-acute-graft-versus-host-disease-and-cytomegalovirus-viremia-after-allogeneic-hematopoietic-stem-cell-transplantation
#20
Xin Yang, Yuanxin Sun, Sudong Zhang, Hui Yang, Jialin Wei, Yi He, Donglin Yang, Erlie Jiang, Mingzhe Han, Xuemei Qin, Sizhou Feng
OBJECTIVES: To analyze the kinetics of T-cell subsets and thymic function reconstitution after allogeneic hematopoietic stem cell transplantation (AHSCT); to determine whether sjTREC (signal joint TCR rearrangement excision circle) and CD31-positive recent thymic emigrant (CD31 + RTE) are correlated with acute graft versus host disease (aGVHD) or CMV (cytomegalovirus) viremia after AHSCT. METHODS: Forty-nine patients who underwent AHSCT in our institution were prospectively enrolled...
May 22, 2017: Hematology (Amsterdam, Netherlands)
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