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Hematology (Amsterdam, Netherlands)

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https://www.readbyqxmd.com/read/28621205/neurocognitive-dysfunction-in-children-with-%C3%AE-thalassemia-major-psychometric-neurophysiologic-and-radiologic-evaluation
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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)
https://www.readbyqxmd.com/read/28506129/kidney-injury-in-infants-and-children-with-iron-deficiency-anemia-before-and-after-iron-treatment
#12
Rasha H Hassan, Shaimaa M Kandil, Mayada S Zeid, Maysaa E Zaki, Ashraf E Fouda
BACKGROUND: Our study aimed to investigate the effects of iron-deficiency anemia (IDA) on renal tubular functions before and after iron treatment for infants and children with IDA. We measured urinary levels of two kidney injury markers: neutrophil gelatinase-associated lipocalin (NGAL) and liver-type fatty acid-binding protein (L-FABP). MATERIAL AND METHODS: Thirty-six infants and children with IDA and 20 matched healthy controls were included. We assessed different laboratory parameters, estimated glomerular filtration rate, urinary levels of NGAL, and L-FABP...
May 16, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28486845/routine-abdominal-ultrasonography-has-limited-value-in-the-care-for-patients-with-indolent-systemic-mastocytosis
#13
C L de Mol, M A W Hermans, R Gerth van Wijk, P M van Hagen, P L A van Daele
OBJECTIVES: Systemic mastocytosis (SM) is a myeloproliferative disease characterized by the accumulation of aberrant mast cells. Since advanced subtypes of SM can lead to organ dysfunction and shortened survival, timely recognition of progressive disease is important for the adequate treatment of SM patients. METHODS: Here, we report the results of our cohort study on the value of routine abdominal ultrasonography for the detection of progression of indolent systemic mastocytosis (ISM)...
May 10, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28486836/inverse-relationship-between-vitamin-d-levels-and-platelet-indices-in-korean-adults
#14
Yon Chul Park, Jin Kim, Min Seok Seo, Sung Won Hong, Eun Seok Cho, Jong-Koo Kim
BACKGROUND AND AIMS: Vitamin D deficiency and increased platelet indices are associated with increased rate or risk of several diseases such as cardiovascular disease and metabolic syndrome, respectively. We investigated whether vitamin D deficiency is associated with increased platelet count (PC) and mean platelet volume (MPV). METHODS AND RESULTS: The study included 3190 subjects older than 20 years. Subjects were divided into three groups based on their vitamin D levels: vitamin D deficiency (<10...
May 10, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28485688/hepatitis-b-reactivation-in-patients-receiving-targeted-therapies
#15
Cheng-Shyong Chang, Chien-Yu Tsai, Sheng-Lei Yan
OBJECTIVES: Hepatitis B virus (HBV) reactivation may occur spontaneously, during or after antiviral therapy, or when receiving immunosuppressive chemotherapy. HBV reactivation has also been reported in cancer patients receiving targeted therapies, such as monoclonal antibody and mammalian target of rapamycin (mTOR) inhibitor. This review article is aimed to discuss the issue regarding chronic HBV reactivation in patients receiving targeted therapies, with a special focus on tyrosine kinase inhibitors...
May 9, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28480800/combined-chemotherapy-for-acute-promyelocytic-leukemia-a-meta-analysis
#16
Xueliang Li, Chao Wang, Guanglong Chen, Buqiang Ji, Yongchang Xu
BACKGROUND: This study evaluates the efficacy of combined chemotherapy for the management of acute promyelocytic leukemia (APL). METHOD: Literature search was carried out in several electronic databases. Meta-analyses were performed to achieve weighted effect sizes of overall survival (OS), disease-free survival (DFS), complete remission (CR) rate, and relapse rate. Metaregression analyses were performed to evaluate the factors affecting CR and relapse rates. RESULTS: Data from 37 studies (7566 patients) were used for meta-analysis...
May 7, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28463078/analysis-of-clinical-characteristics-and-prognostic-factors-of-multiple-myeloma-a-retrospective-single-center-study-of-787-cases
#17
Jiejing Qian, Jie Jin, Hong Luo, Chunji Jin, Lei Wang, Wenbin Qian, Haitao Meng
OBJECTIVE: This study aims to explore the clinical features of multiple myeloma (MM) and the influence of various prognostic factors on survival. METHODS: A retrospective analysis, consisting of clinical characteristics analysis and laboratory examinations, was performed on 787 MM patients. Clinical and laboratory parameters were analyzed by multivariate process and compared across different groups. RESULTS: Of the 787 patients enrolled (median age, 61 years old, range 29-89 years old), 491 (62...
May 2, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28447549/a-child-with-severe-iron-deficiency-anemia-and-a-complex-tmprss6-genotype
#18
Anna Paola Capra, Elisa Ferro, Laura Cannavò, Maria Angela La Rosa, Giuseppina Zirilli
OBJECTIVES: We report a case of a 7-year-old girl with severe hypochromic microcytic anemia, who was unresponsive to classical iron supplements. We suspected IRIDA, iron-refractory iron-deficiency anemia, a genetic iron metabolism disorder, caused by TMPRSS6 variations. TMPRSS6 encodes matriptase-2, a negative regulator of hepcidin, and its pathological variants are related to normal to high levels of hepcidin. We analyzed the TMPRSS6 gene and we improved clinical management of the patient, selecting the appropriate supplementation therapy...
April 27, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28441921/acquired-hemophilia-a-a-review-of-recent-data-and-new-therapeutic-options
#19
Massimo Franchini, Stefania Vaglio, Giuseppe Marano, Carlo Mengoli, Sara Gentili, Simonetta Pupella, Giancarlo Maria Liumbruno
OBJECTIVES: Acquired hemophilia A (AHA) is a rare, but potentially life-threatening, bleeding disorder caused by an autoantibody against factor VIII that interferes with its coagulant function. METHODS: We performed a narrative review focusing on the diagnostic aspects of AHA and on the current treatment strategies with particular regard to new data and therapeutic developments. RESULTS: The management of this severe hemorrhagic disorder is based on the control of bleeding with the use of bypassing agents and on the utilization of a variety of immunosuppressant agents with the goal of eliminating the autoantibody permanently...
April 25, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28441911/the-effects-of-a-data-driven-maximum-surgical-blood-ordering-schedule-on-preoperative-blood-ordering-practices
#20
C L Woodrum, M Wisniewski, D J Triulzi, J H Waters, L H Alarcon, M H Yazer
OBJECTIVES: The maximum surgical blood ordering schedule (MSBOS) provides guidelines for pre-operative pre-transfusion testing for elective surgical procedures. This study compared blood ordering and utilization during the period when the MSBOS was created by achieving consensus between the blood bank and the various surgical specialties, and after the introduction of an MSBOS created by using department-specific red blood cell (RBC) transfusion data (data driven MSBOS, dMSBOS). METHODS: The dMSBOS was created by analyzing 12 months of RBC transfusion data for each procedure across a regional health system...
April 25, 2017: Hematology (Amsterdam, Netherlands)
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