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European Journal of Haematology

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https://www.readbyqxmd.com/read/28926144/characterization-of-tp53-mutations-in-low-grade-myelodysplastic-syndromes-and-myelodysplastic-syndromes-with-a-non-complex-karyotype
#1
Wei Wang, Mark J Routbort, Zhenya Tang, Chi Young Ok, Keyur P Patel, Naval Daver, Guillermo Garcia-Manero, L Jeffrey Medeiros, Sa A Wang
OBJECTIVES: Although commonly associated with high-grade myelodysplastic syndrome (MDS) and MDS with a complex karyotype, TP53 mutations also occur in low-grade MDS and MDS with a non-complex karyotype. In latter cases, their clinicopathological features and the characteristics of TP53 mutations remain poorly characterized. METHODS: 176 MDS cases with TP53 mutations were stratified and characterized based on their karyotype and histologic subtype. RESULTS: Among 176 cases, 17% had a non-complex karyotype and 24% were low-grade MDS...
September 19, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28905428/cost-effective-screening-of-dnmt3a-coding-sequence-identifies-somatic-mutation-in-pediatric-t-cell-acute-lymphoblastic-leukemia
#2
Bronisława Szarzyńska-Zawadzka, Maria Kosmalska, Łukasz Sędek, Alicja Sonsala, Magdalena Twardoch, Jerzy R Kowalczyk, Tomasz Szczepański, Michał Witt, Małgorzata Dawidowska
BACKGROUND AND OBJECTIVES: In pediatric T-cell acute lymphoblastic leukemia (T-ALL) risk assignment schemes preclude reliable prediction of outcome and thus new prognostic factors are needed. Mutations in DNMT3A are candidate prognostic and classification markers in adults with acute myeloid leukemia (AML) and T-ALL and thus were considered as candidates prognostic markers in pediatric T-ALL. PATIENTS AND METHODS: DNMT3A mutational status was investigated in 74 pediatric T-ALL samples collected at diagnosis...
September 14, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28895206/in-patient-versus-out-patient-autologous-hematopoietic-stem-cell-transplantation-for-multiple-myeloma
#3
Nina Shah, A Megan Cornelison, Rima Saliba, Sairah Ahmed, Yago L Nieto, Qaiser Bashir, Simrit Parmar, Chitra Hosing, Uday Popat, Elizabeth J Shpall, Richard E Champlin, Muzaffar Qazilbash
BACKGROUND: High dose chemotherapy and autologous hematopoietic stem cell transplantation (auto-HCT) is commonly performed for multiple myeloma (MM) patients and may be as safe in the out-patient setting as in the in-patient setting. METHODS: We performed a single-center retrospective analysis of all MM patients undergoing auto-HCT between January 2008 - December 2012. We categorized patients as out-patient versus in-patient auto-HCT and compared clinical characteristics and outcomes between the groups...
September 12, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28895203/rapid-reduction-of-bcr-abl1-transcript-predicts-deep-molecular-response-in-dasatinib-treated-chronic-phase-chronic-myeloid-leukaemia-patients
#4
Kazunori Murai, Kohei Yamaguchi, Shigeki Ito, Takuto Miyagishima, Motohiro Shindo, Kentaro Wakasa, Mitsue Inomata, Takahiro Nagashima, Takeshi Kondo, Nozomu Fujimoto, Satoshi Yamamoto, Masakatsu Yonezumi, Tatsuo Oyake, Kowata Shugo, Yasuhiko Tsukushi, Takahiro Mine, Kuniaki Meguro, Kazuhiko Ikeda, Reiko Watanabe, Souichi Saito, Shinji Sato, Katsushi Tajima, Takaaki Chou, Kohmei Kubo, Koji Oba, Junichi Sakamoto, Yoji Ishida
OBJECTIVES: We conducted a phase-II study to evaluate the efficacy and safety of dasatinib in patients newly diagnosed with chronic-phase chronic myeloid leukaemia (CML-CP) in Japan (IMIDAS PART 2 study). METHODS: Seventy-nine patients were administered 100 mg dasatinib once daily. We examined pre-treatment and post-treatment influences of various factors. The BCR-ABL1 international scale (IS), halving time (HT), and reduction rate of BCR-ABL1 transcript within the initial 1 or 3 months of therapy (RR-BCR-ABL11m,3m ) were the post-treatment factors investigated to predict the molecular response...
September 12, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28888044/novel-mutations-in-thai-patients-with-glanzmann-thrombasthenia
#5
Rungnapa Ittiwut, Pintip Suchartlikitwong, Yaowaree Kittikalayawong, Chupong Ittiwut, Karan Prasopsanti, Darintr Sosothikul, Vorasuk Shotelersuk, Kanya Suphapeetiporn
OBJECTIVES: Glanzmann thrombasthenia (GT) is an autosomal recessive platelet disorder, caused by defects of the platelet integrin αIIbβ3 (GPIIb/IIIa) resulting from pathogenic mutations in either ITGA2B or ITGB3. It is characterized by spontaneous mucocutaneous bleeding. The molecular features of GT in Thailand have not been identified. This study aimed to determine the clinical and molecular features of unrelated Thai patients with GT. METHODS: Four patients with clinically suspected GT were recruited at the Division of Pediatric Hematology/Oncology, King Chulalongkorn Memorial Hospital...
September 9, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28888029/expression-of-dna-repair-genes-are-important-molecular-findings-in-cd34-stem-cells-of-myelodysplastic-sydrome
#6
LETTER
Howard L Ribeiro Junior, Allan Rodrigo Soares Maia, Roberta Taiane G de Oliveira, Antônio Wesley Araújo Dos Santos, Marília Braga Costa, Izabelle Rocha Farias, Daniela de Paula Borges, Silvia Maria M Magalhães, Ronald F Pinheiro
We read with great interest the study of Valka et al. [1], recently published and we would like to reinforce the importance of DNA repair genes in the pathogenesis of myelodysplastic syndrome (MDS). The authors monitored and detected a differential expression of panel containing 84 DNA repair-associated genes in samples of CD34+ cells of MDS patients [1]. Here, we would like to complement the Valka et al. [1] study with our new recent analyzes of gene expression in a panel of 14 DNA repair genes (ATM, BRCA1, BRCA2, RAD51, XRCC5, XRCC6, LIG4, XPA, XPC, XPD/ERCC2, XPF/ERCC4, XPG/ERCC5, CSA/ERCC8 and CSB/ERCC6) in CD34+ stem cell samples, using quantitative PCR methodologies, and it's relation with clinical laboratory variables of Brazilian MDS patients...
September 9, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28888028/effect-of-reduced-intensity-conditioning-and-the-risk-of-late-onset-non-infectious-pulmonary-complications-in-pediatric-patients
#7
Masayuki Nagasawa, Noriko Mitsuiki, Yuki Aoki, Toshiaki Ono, Takeshi Isoda, Kohsuke Imai, Masatoshi Takagi, Michiko Kajiwara, Hirokazu Kanegane, Tomohiro Morio
OBJECTIVE: Late-onset non-infectious pulmonary complications (LONIPCs) contribute to higher morbidity and mortality after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Therefore, we investigated the risk factors of LONIPCs in pediatric patients. METHOD: Between 2001 and 2011, 74 pediatric patients (range, 7 months to 22.7 years old; median 6.5 years old), including 29 with a primary immunodeficiency underwent 80 allo-HSCTs at our institution. Sixty-seven patients who survived more than 3 months after allo-HSCT were analyzed retrospectively...
September 9, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28888027/dermatopathic-lymphadenopathy-with-langerhans-cell-chimerism-in-graft-versus-host-disease-of-the-skin
#8
Andreas Kreft, Kristina Krümpelmann, Andreas Rosenwald, David Hollemann, Eva Maria Wagner-Drouet
Dermatopathic lymphadenopathy (DL) is well-known in inflammatory skin disease; however, it has not been reported in graft-versus-host disease (GvHD) after allogeneic stem cell transplantation. Here, we report two cases of DL in patients with acute GvHD of the skin and demonstrate complete donor chimerism of Langerhans cells within the lymph nodes. This article is protected by copyright. All rights reserved.
September 9, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28886236/waldenstr%C3%A3-m-s-macroglobulinemia-two-malignant-clones-in-a-monoclonal-disease-molecular-background-and-clinical-reflection
#9
REVIEW
K Growková, E Kryukova, Z Kufová, J Filipová, T Ševčíková, L Říhová, M Kaščák, F Kryukov, R Hájek
Waldenström's macroglobulinemia (WM) is a complex disease characterized by apparent morphological heterogeneity within the malignant clonal cells representing a continuum of small lymphocytes, plasmacytoid lymphocytes, and plasma cells. At the molecular level, the neoplastic B-cell derived clone has undergone somatic hypermutation, but not isotype switching, and retains the capability of plasmacytic differentiation. Although by classical definition, WM is formed by monoclonal expansion, long-lived clonal B lymphocytes are of heterogeneous origin...
September 8, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28886228/the-serum-heavy-light-chain-immunoassay-a-valuable-tool-for-sensitive-paraprotein-assessment-risk-and-disease-monitoring-in-monoclonal-gammopathies
#10
Christine Greil, Gabriele Ihorst, Felix Gaiser, Ulrich Salzer, Emanuel Bisse, Efstathios Kastritis, Heinz Ludwig, Ralph Wäsch, Monika Engelhardt
OBJECTIVE: The heavy/light chain (HLC)-immunoassay quantifies light chain types of each immunoglobulin class in patients with monoclonal gammopathies. METHODS: We assessed 147 consecutive patients with different forms and stages of plasma cell dyscrasias (PCD) who received standard tests (serum and urine protein electrophoresis [SPEP, UPEP], immunofixation [IFE], serum free light chain [SFLC]) and HLC-immunoassay. Patients with multiple myeloma (MM, n=102), smoldering MM (SMM, n=5), monoclonal gammopathy of undetermined significance (MGUS, n=28) and waldenström's macroglobulinemia (WM, n=12) were included...
September 8, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28885736/prevalence-and-prognostic-impact-of-anemia-and-iron-deficiency-in-patients-hospitalized-in-an-internal-medicine-ward-the-pro-iron-study
#11
Cândida Fonseca, Manuel Araújo, Patrícia Moniz, Filipa Marques, Inês Araújo, Luís Costa, Joana Rodrigues, Luciana Frade, Arturo Botella, Susana Jesus, Ana Leitão, Luís Campos
To assess prevalence, predictive factors, and prognostic impact on in-hospital mortality of anemia, iron deficiency anemia (IDA), iron deficiency with or without anemia (ID), and iron deficiency without anemia (IDWA) in patients admitted to an internal medicine ward METHODS: This one-year prospective study collected data on demographics, medical history, and blood tests in 771 consecutive patients on admission RESULTS: Most patients were ≥65 years old (80%), and had hypertension (63%), moderate chronic kidney disease (CKD) (43%), and heart failure (41%)...
September 8, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28885719/post-transplant-consolidation-plus-lenalidomide-maintenance-vs-lenalidomide-maintenance-alone-in-multiple-myeloma-a-systematic-review
#12
REVIEW
Fatimah Al-Ani, Martha Louzada
BACKGROUND: In newly diagnosed multiple myeloma (NDMM), autologous stem cell transplantation (ASCT) remains the standard approach for transplant-eligible patients. To control the inevitable relapse, post-transplant consolidation/maintenance strategies are commonly used. However, the benefit of post-transplant consolidation is still uncertain. METHOD: We conducted a systematic review of phase II/III studies to compare the efficacy of post-ASCT consolidation plus lenalidomide maintenance (CON+LEN) vs...
September 8, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28885714/monoclonal-gammopathy-of-undetermined-significance-mgus-as-viewed-by-haematology-healthcare-professionals
#13
Charlene M McShane, Blain Murphy, Kah Heng Lim, Lesley A Anderson
OBJECTIVES: To investigate the words and descriptions used by haematology healthcare professionals (HCPs) to describe monoclonal gammopathy of undetermined significance (MGUS) to their patients. METHODS: A cross-sectional survey of haematology HCPs attending an annual haematology conference was undertaken. Content analysis was applied to the returned qualitative responses. RESULTS: In total, 55 people, many of whom were doctors (n=32; 58.2%) responded...
September 8, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28884875/successful-collection-of-peripheral-blood-stem-cells-upon-vide-chemomobilization-in-sarcoma-patients
#14
Katharina Kriegsmann, Christoph Heilig, Martin Cremer, Philipp Novotny, Mark Kriegsmann, Thomas Bruckner, Carsten Müller-Tidow, Gerlinde Egerer, Patrick Wuchter
OBJECTIVES: In patients with Ewing sarcoma and some distinct subgroups of soft tissue sarcoma (STS), a quantitatively sufficient autologous peripheral blood stem cell (PBSC) collection for stem cell support might facilitate treatment continuation, dose-intensification and high-dose chemotherapy. Here we provide a detailed evaluation of PBSC collection upon vincristine, ifosfamide, doxorubicin, and etoposide (VIDE) chemomobilization. METHODS: Mobilization and collection parameters of 42 sarcoma patients (Ewing sarcoma n=35, other STS n=7) were analyzed retrospectively...
September 8, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28881484/primary-cells-in-bcr-fgfr1-positive-8p11-myeloproliferative-syndrome-are-sensitive-to-dovitinib-ponatinib-and-dasatinib
#15
Niklas Landberg, Arta Dreimane, Marianne Rissler, Rolf Billström, Helena Ågerstam
OBJECTIVES: Translocations involving the Fibroblast growth factor receptor 1 (FGFR1) gene are associated with the 8p11 myeloproliferative syndrome (EMS), a rare neoplasm that following a usually short chronic phase progresses into acute myeloid or lymphoid leukemia. The treatment commonly involves chemotherapy and, if possible, allogeneic stem cell transplantation which is the only therapeutic option for long term survival. Given the aggressive course of EMS, we here evaluated tyrosine kinase inhibitors as treatment options to delay disease progression...
September 7, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28850718/the-management-of-hodgkin-lymphomas-in-pregnancies
#16
REVIEW
Y Moshe, O S Bentur, M Lishner, I Avivi
Hodgkin lymphoma is the most common hematological malignancy in pregnancy. Its management presents several unique challenges, as decisions have to take both maternal and fetal risks into consideration. Using three hypothetical cases we review current evidence and guidelines, and suggest our recommendations for managing pregnant Hodgkin lymphoma patients. The opportunity for a prompt and accurate diagnosis should not be missed; this may be achieved by vigilance to suggestive symptoms, performance of biopsy which is not contraindicated during pregnancy and use of MRI for staging...
August 29, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28850716/thalidomide-induces-haematologic-responses-in-patients-with-%C3%AE-thalassaemia
#17
Jiangming Chen, Weijian Zhu, Ning Cai, Shengdi Bu, Jinyan Li, Lan Huang
OBJECTIVE: This study aimed to evaluate the clinical and laboratory outcomes of a group of patients with β-thalassaemia major or intermedia treated with thalidomide. METHODS: Clinical and laboratory data were obtained by analysing medical records of β-thalassaemia patients. Using the self-control method, the levels of haemoglobin (Hb), foetal haemoglobin (HbF), white blood cells (WBC), and platelets were detected before and after the treatment, and adverse reactions were recorded...
August 29, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28833682/preliminary-report-measurement-of-apixaban-and-rivaroxaban-in-plasma-from-bleeding-patients
#18
Kathrine O F Jensen, Svend H Hansen, Jens P Goetze, Amalie Jesting, Jakob Stensballe, Henrik Hansen
OBJECTIVES: The direct oral anticoagulants (DOACs) apixaban and rivaroxaban are used for anticoagulation treatment. Although biochemical monitoring is not required, severe bleedings caused by DOAC have been reported. We therefore evaluated the chromogenic assay Biophen DiXaI(®) (Biophen) using plasma from non-bleeding patients in treatment with DOAC and on spiked plasma from patients with ongoing bleeding. METHODS: The Biophen method was compared with an in-house reference method; liquid chromatography-tandem mass spectrometry (LC-MS/MS) using plasma spiked with the compounds ranging from 20-1500 μg/L...
August 21, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28833529/distress-and-unmet-needs-during-treatment-and-quality-of-life-in-early-cancer-survivorship-a-longitudinal-study-of-haematological-cancer-patients
#19
Devesh Oberoi, Victoria M White, John F Seymour, H Miles Prince, Simon Harrison, Michael Jefford, Ingrid Winship, David J Hill, Damien Bolton, Jeremy Millar, Nicole Wong Doo, Anne Kay, Graham Giles
OBJECTIVE: To examine the influence of anxiety, depression and unmet supportive care needs on future quality of life (QoL) in multiple myeloma (MM) and diffuse large B cell lymphoma (DLBCL) patients. METHODS: MM and DLBCL patients recruited through the population-based Victorian Cancer Registry. Data was collected through two telephone interviews: T1) on average 7 months post diagnosis, T2) average 8 months later. QoL was examined at T2 using the Functional Assessment of Cancer Therapy (FACT-G) scale...
August 21, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28833639/expression-and-significance-of-pim-3-kinase-in-adult-t-cell-leukemia
#20
Chie Ishikawa, Masachika Senba, Tadashi Hashimoto, Atsushi Imaizumi, Naoki Mori
BACKGROUND: Human T-cell leukemia virus type 1 (HTLV-1) causes adult T-cell leukemia (ATL). Viral Tax protein plays a major role in ATL development. Pim family of serine/threonine kinases is composed of Pim-1, -2, and -3. The potential of Pim family as a target in ATL was analyzed. METHODS: RT-PCR and Western blotting were used to determine the expression of Pim kinases, Tax, and intracellular signal molecules. Knockdown of Pim-3 and RelA was performed using small interfering RNA...
August 19, 2017: European Journal of Haematology
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