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Hematology (Amsterdam, Netherlands)

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https://www.readbyqxmd.com/read/28447549/a-child-with-severe-iron-deficiency-anemia-and-a-complex-tmprss6-genotype
#1
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
#2
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
#3
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)
https://www.readbyqxmd.com/read/28415913/characteristics-and-management-of-primary-and-other-immune-thrombocytopenias-spanish-registry-study
#4
Javier Palau, Esther Sancho, Magdalena Herrera, Sol Sánchez, María Eva Mingot, Rosa Isabel Upegui, Mª José Rodríguez Salazar, Fátima de la Cruz, Mª Cristina Fernández, Tomás José González López, José Julio Hernández, Eduardo Ríos, Mª Fernanda López-Fernández, Marta García, José-Ángel Hernández, Miguel A Sanz
BACKGROUND: The natural history and its modulation by treatments administered for immune thrombocytopenia (ITP) in the clinical practice remains unknown. In addition, little information is available on the characteristics and management of ITP in Spain. METHODS: We conducted an observational, multicenter, registry in 70 Hematology Services from Spain between 2009 and 2011, which included children from 2 months of age and adults with primary ITP or another ITP diagnosed within the last 6 months (platelet count [PC] < 100 × 10(9)/l)...
April 17, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28413914/prognostic-value-of-the-soluble-interleukin-2-receptor-level-after-patients-with-follicular-lymphoma-achieve-a-response-to-r-chop
#5
Kento Umino, Shin-Ichiro Fujiwara, Takashi Ikeda, Yumiko Toda, Shoko Ito, Kiyomi Mashima, Daisuke Minakata, Hirofumi Nakano, Ryoko Yamasaki, Yasufumi Kawasaki, Miyuki Sugimoto, Chihiro Yamamoto, Masahiro Ashizawa, Kaoru Hatano, Kazuya Sato, Iekuni Oh, Ken Ohmine, Kazuo Muroi, Yoshinobu Kanda
OBJECTIVES: Follicular lymphoma (FL) is a clinically and biologically heterogeneous disease. Therefore, it is important to identify factors that can predict its clinical outcome. METHODS: We retrospectively evaluated the usefulness of soluble interleukin-2 receptor (sIL-2R) levels after R-CHOP (posttreatment sIL-2R) in 72 patients with newly diagnosed FL who had either a complete response (CR) or partial response. With the use of a recursive partitioning analysis, we determined the cut-off values of post- and pretreatment sIL-2R levels that were associated with disease progression, which corresponded to 486...
April 17, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28413901/elevation-of-cd16-cd56-nk-cells-and-down-regulation-of-serum-interleukin-21-il-21-and-il-1%C3%AE-after-splenectomy-in-relapsed-hemophagocytic-lymphohistiocytosis-of-unknown-cause
#6
Jingshi Wang, Wei Han, Zhuo Gao, Yini Wang, Lin Wu, Jia Zhang, Wenyuan Lai, Zhao Wang
OBJECTIVES: Encouraging progress has been made in application of splenectomy in the treatment of relapsed hemophagocytic lymphohistiocytosis (HLH) of unknown cause. The aim was to determine the roles of lymphocyte subpopulations and inflammatory cytokines in splenectomy. METHODS: We retrospectively analyzed changes in lymphocyte subpopulations and levels of inflammatory cytokines at different time-points before and after splenectomy in the patients with relapsed HLH of unknown cause, as well as the correlations between these changes and the disease prognosis...
April 15, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28406068/clinical-and-hematological-relevance-of-jak2-v617f-and-calr-mutations-in-bcr-abl-negative-et-patients
#7
N Limsuwanachot, B Rerkamnuaychoke, S Chuncharunee, T Pauwilai, R Singdong, P Rujirachaivej, T Chareonsirisuthigul, T Siriboonpiputtana
BACKGROUND: Classical BCR-ABL1-negative myeloproliferative neoplasms (MPNs) including polycythemia vera, essential thrombocythemia (ET), and primary myelofibrosis frequently harbor JAK2, MPL, and CALR somatic mutations. METHODS: AS-PCR for JAK2 V617F, pyrosequencing for MPL W515L/K, and PCR-fragment analysis for CALR exon 9 mutations were established to analyze genomic DNA isolated from peripheral blood samples of 58 newly diagnosed ET patients in Thailand. RESULTS: JAK2 V617F was detected in 41 patients (71%) and CALR exon 9 mutation was positive in eight patients (14%), whereas no mutation of MPL W515L/K was observed in this study...
April 13, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28399703/value-of-speckle-tracking-echocardiography-for-detection-of-clinically-silent-left-ventricular-dysfunction-in-patients-with-%C3%AE-thalassemia
#8
Mozhgan Parsaee, Sedigheh Saedi, Pegah Joghataei, Azita Azarkeivan, Zahra Alizadeh Sani
OBJECTIVE: β-Thalassemia is an inherited hemoglobin disorder resulting in chronic hemolytic anemia requiring chronic transfusion therapy. Cardiac involvement is the main cause of death in patients with thalassemia major. The narrow border is between overt myocardial dysfunction and clinically silent left ventricular (LV) dysfunction in patients with thalassemia. Therefore, we need novel parameters in different imaging techniques to discover cardiac involvement in an early and subtle stage...
April 12, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28397608/clinical-usefulness-of-fdg-pet-ct-for-the-evaluation-of-various-types-of-adult-t-cell-leukemia
#9
Sawako Nakachi, Masahiro Okada, Satoko Morishima, Yurika Agarie, Sakiko Kitamura, Sachie Uchibori, Shouhei Tomori, Taeko Hanashiro, Natsuki Shimabukuro, Keita Tamaki, Iori Tedokon, Kazuho Morichika, Yukiko Nishi, Takeaki Tomoyose, Kennosuke Karube, Takuya Fukushima, Sadayuki Murayama, Hiroaki Masuzaki
PURPOSE: The aim was to explore undefined useful indices for clinically grading adult T-cell leukemia (ATL) using [(18)F] 2-fluoro-2-deoxyglucose (FDG) - positron emission tomography/computed tomography (PET/CT). METHODS: A total of 28 patients with ATL (indolent, 9; aggressive, 19) were enrolled; all patients with aggressive ATL underwent FDG-PET/CT before chemotherapy. Patients with indolent ATL underwent FDG-PET/CT at the time of suspected disease progression and/or transformation; some received lymph node biopsy...
April 11, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28395594/upregulation-of-adam12-contributes-to-accelerated-cell-proliferation-and-cell-adhesion-mediated-drug-resistance-cam-dr-in-non-hodgkin-s-lymphoma
#10
Haibing Yin, Fei Zhong, Yu Ouyang, Qiru Wang, Linlin Ding, Song He
OBJECTIVE: ADAM12 is a member of a disintegrin and metalloproteinase family and has been reported to participate in the development of variety of tumors. However, the role of ADAM12 in Non-Hodgkin Lymphoma (NHL) has not been investigated. The present study was undertaken to determine the expression and biologic function of ADAM12 in human NHL. METHODS: First, we constructed a model of cell adhesion in NHL, the mRNA, and protein level of ADAM12 in suspension and the adhesion model was analyzed by RT-PCR and western blot...
April 10, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28385068/transforming-growth-factor-15-increased-in-severe-aplastic-anemia-patients
#11
Yuanyuan Shao, Honglei Wang, Chunyan Liu, Qiuying Cao, Rong Fu, Huaquan Wang, Ting Wang, Weiwei Qi, Zonghong Shao
OBJECTIVES: The patients with severe aplastic anemia (SAA) usually rely on red cell transfusion which lead to secondary iron overload. Transforming growth differentiation factor-15 (GDF-15) plays an important role in erythropoiesis and iron regulation. In this study, we investigated the level of GDF-15 and other indexes of iron metabolism in SAA patients to explore the correlation with GDF-15 and iron overload in SAA. METHODS: The levels of serum GDF-15, hepcidin (Hepc), and erythropoietin (EPO) were determined by ELISA...
April 6, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28327053/predictive-parameters-for-imatinib-failure-in-patients-with-chronic-myeloid-leukemia
#12
Danijela Lekovic, Mirjana Gotic, Natasa Milic, Biljana Zivojinovic, Jelica Jovanovic, Natasa Colovic, Violeta Milosevic, Andrija Bogdanovic
OBJECTIVE: Until recently, imatinib was the standard first-line treatment in chronic myeloid leukemia (CML). The inclusion of nilotinib and dasatinib as first-line options in CML raised a debate on treatment selection. The aim of our study was to analyze predictive parameters for imatinib response as the first-line treatment of CML patients. METHODS: The study included 168 consecutive patients with chronic phase Philadelphia-positive CML who were diagnosed and treated with Imatinib 400 mg once daily at a single university hospital...
March 22, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28300523/assessment-of-th17-treg-cells-and-th-cytokines-in-an-improved-immune-thrombocytopenia-mouse-model
#13
Guoyang Zhang, Ping Zhang, Hongyun Liu, Xiaoyan Liu, Shuangfeng Xie, Xiuju Wang, Yudan Wu, Jianxing Chang, Liping Ma
OBJECTIVES: The improved passive immune thrombocytopenia (ITP) mouse model has been extensively utilized for the study of ITP. However, how closely this model matches the human inflammation state and immune background is unclear. Our study aimed to explore the profile of Th cytokines and Th17/Treg cells in the model. METHODS: We induced the ITP mouse model by dose-escalation injection of MWReg30. The serum levels of cytokines (IFN-γ, IL-2, IL-4, IL-10, IL-17A, and TGF-β1) were measured by enzyme-linked immunosorbent assay and the frequency of Th17 and Treg cells was measured by flow cytometry...
March 16, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28292214/adamtsl5-and-cdh11-putative-epigenetic-markers-for-therapeutic-resistance-in-acute-lymphoblastic-leukemia
#14
Maha Abdullah, Chee Wei Choo, Hamidah Alias, Eni Juraidah Abdul Rahman, Hishamshah Mohd Ibrahim, Rahman Jamal, Noor Hamidah Hussin
BACKGROUND AND OBJECTIVES: DNA hypermethylation has been linked to poor treatment outcome in childhood acute lymphoblastic leukemia (ALL). Genes differentially methylated in the chemoresponsive pre-B-ALL compared to chemoresistant pre-B-ALL cases provide potential prognostic markers. METHODS: DNA methylation profiles of five B-ALL childhood patients who achieved morphological complete remission (chemoresponsive) and five B-ALL patients who did not (chemoresistant) after induction treatments as well as four normal controls were compared on 27 000 CpG sites microarray chips...
March 15, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28281408/the-role-of-follicular-t-helper-cells-in-patients-with-malignant-lymphoid-disease
#15
Dong-Ming Zhou, Yan-Xia Xu, Li-Ying Zhang, Yu Sun, Zi-Yan Wang, Yu-Qing Yuan, Jin-Xiang Fu
OBJECTIVES: To investigate the dynamic change of follicular T helper cells (TFH) in patients with malignant lymphoid disease (MLD) and to explore its clinical significance. METHODS: The dynamic change of TFH cells, ICOS(+)- and PD-1(+) TFH cells at pretreatment and different treatment periods was determined by flow cytometry in 85 MLD patients. Concentration of interleukin 21 (IL-21) was evaluated by ELISA, and the correlation between clinical prognosis and the ratio of TFH cells was analyzed...
March 10, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28271814/gata2-null-mutation-associated-with-incomplete-penetrance-in-a-family-with-emberger-syndrome
#16
Aniel Jessica Leticia Brambila-Tapia, José Elías García-Ortiz, Pascal Brouillard, Ha-Long Nguyen, Miikka Vikkula, Blanca Estela Ríos-González, Roberto de Jesús Sandoval-Muñiz, Ana Karen Sandoval-Talamantes, Lucina Bobadilla-Morales, Jorge Román Corona-Rivera, Lisette Arnaud-Lopez
INTRODUCTION: GATA2 mutations are associated with several conditions, including Emberger syndrome which is the association of primary lymphedema with hematological anomalies and an increased risk for myelodysplasia and leukemia. OBJECTIVE: To describe a family with Emberger syndrome with incomplete penetrance. METHODS: A DNA sequencing of GATA2 gene was performed in the parents and offspring (five individuals in total). RESULTS: The family consisted of 5 individuals with a GATA2 null mutation (c...
March 8, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28253825/upregulation-of-microrna-21-is-a-poor-prognostic-marker-in-patients-with-childhood-b-cell-acute-lymphoblastic-leukemia
#17
Hany Abedelmalik Labib, Neveen G Elantouny, Nevin F Ibrahim, Ahmed A Alnagar
OBJECTIVES: Many studies have demonstrated that microRNA-21 (miR-21) is an oncogene and is upregulated in tumor tissue. However, its association with B-cell acute lymphoblastic leukemia (B-ALL) remains poorly understood. METHODS: The expression of miR-21 was detected by real-time quantitative PCR in 75 children with de novo B-ALL as well as in 50 healthy controls. This study was conducted to evaluate the miR-21 as a biomarker for risk assessment, diagnosis and prognosis...
March 2, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28228042/haemoglobin-f-a2-and-s-levels-in-subjects-with-or-without-sickle-cell-trait-in-south-eastern-gabon
#18
Landry-Erik Mombo, Gaël Mabioko-Mbembo, Roland-Fabrice Kassa-Kassa, Emmanuel Ontsitsagui, Statiana Mboui-Ondo, Leatitia Nzé-Kamsi, Dieudonné Nkoghé, Jacques Elion
BACKGROUND: Infant mortality due to sickle cell disease in sub-Saharan Africa is high, necessitating a better understanding of the modulating factors of the disease in this region. METHODS: We assessed the hereditary persistence of foetal haemoglobin and α-thalassemia. We diagnosed 787 subjects, with or without sickle cell trait, by capillary electrophoresis in the Medical Diagnostic Laboratory of the CIRMF (Franceville, Gabon). RESULTS: Heterocellular and pancellular forms of hereditary persistence of foetal haemoglobin occurred at low rates of 10...
February 23, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28220719/protector-effect-of-%C3%AE-thalassaemia-on-cholecystitis-and-cholecystectomy-in-sickle-cell-disease
#19
Robéria M Pontes, Elaine S Costa, Patrícia F R Siqueira, Jussara F F Medeiros, Andréa Soares, Fabiana V de Mello, Maria C Maioli, Isaac L S Filho, Liliane R Alves, Marcelo G P Land, Marcos K Fleury
OBJECTIVES: Cholecystitis is one of the complications of symptomatic cholelithiasis responsible for high levels of morbidity of sickle cell disease (SCD) patients. Here, we investigated the possible protective role of single gene deletions of α-thalassaemia in the occurrence of cholelithiasis and cholecystitis in SCD patients, as well as the cholecystectomy requirements. METHODS: The α-globin genotype was determined in 83 SCD patients using the multiplex-polymerase chain reaction and compared with clinical events...
February 21, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28218017/molecular-and-clinical-analysis-of-haemoglobin-lepore-in-campania-a-region-of-southern-italy
#20
Paolo Ricchi, Massimiliano Ammirabile, Anna Spasiano, Silvia Costantini, Tiziana Di Matola, Patrizia Cinque, Caterina Saporito, Aldo Filosa, Leonilde Pagano
OBJECTIVE: To date in Italy, there is paucity on data about the prevalence, clinical and haematological features of patients carrying the haemoglobin (Hb) Lepore variant in homozygous or in association with other haemoglobinopathies. METHODS: Here we report the results of a retrospective analysis on 33 patients from Campania, a region of Southern Italy, historically followed at 'UOSD Malattie Rare del Globulo Rosso' of Cardarelli hospital, Naples, Italy. RESULTS: We described 33 patients carrying the Hb Lepore variant: 21 compound heterozygotes with a common thalassaemia allele, six patients with homozygous state for Hb Lepore, five patients with Hb Lepore/Hb S and one patient with Hb Lepore/Hb Neapolis were identified...
February 20, 2017: Hematology (Amsterdam, Netherlands)
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