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European Journal of Haematology

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https://www.readbyqxmd.com/read/29791053/impact-of-ruxolitinib-pretreatment-on-outcomes-after-allogeneic-stem-cell-transplantation-in-patients-with-myelofibrosis
#1
Sharifah Shahnaz Syed Abd Kadir, Maximilian Christopeit, Wulf Gerald, Eva Wagner, Martin Bornhauser, Thomas Schroeder, Martina Crysandt, Karin Mayer, Julia Jonas, Matthias Stelljes, Anita Badbaran, Francis Ayuketang Ayuk, Ioanna Triviai, Dominik Wolf, Christine Wolschke, Nicolaus Kröger
INTRODUCTION: Ruxolitinib is the first approved drug for treatment of myelofibrosis, but its impact of outcome after allogeneic stem cell transplantation (ASCT) is unknown. PATIENTS AND METHODS: We reported on 159 myelofibrosis patients (pts) with a median age of 59 years (r: 28 - 74) who received reduced intensity ASCT between 2000 and 2015 in 8 German centers from related (n = 23), matched (n=86) or mismatched (n=50) unrelated donors. Forty-six (29%) patients received ruxolitinib at any time point prior to ASCT...
May 23, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29791043/infection-with-ibrutinib-in-patients-with-chronic-lymphocytic-leukemia-cll-how-strong-is-the-association
#2
LETTER
Somedeb Ball, Wasawat Vutthikraivit, Abhishek Maiti, Nicholas J Short
We read the recent review by Tillman et al. [1] with great interest and wanted to revisit the association of ibrutinib with increased risk of infection. Based on our clinical experience and the results of ibrutinib trials, we agree with the authors regarding the possibility of increased risk of infections with ibrutinib. [2,3] However, chronic lymphocytic leukemia (CLL) and other B-cell malignancies by themselves confer increased risk of infections owing to acquired defects of the innate and adaptive immunity secondary to the disease process, hypogammaglobulinemia, and T-cell and complement dysfunction, among others...
May 23, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29786897/targeted-next-generation-sequencing-for-the-diagnosis-of-patients-with-rare-congenital-anemias
#3
Noa Shefer Averbuch, Orna Steinberg-Shemer, Orly Dgany, Tanya Krasnov, Sharon Noy-Lotan, Joanne Yacobovich, Amir A Kuperman, Antonis Kattamis, Ayelet Ben Barak, Batia Roth-Jelinek, Evgeni Chubar, Evelyn Shabad, Gustavo Dufort, Martin Ellis, Ofir Wolach, Idit Pazgal, Abed Abu Quider, Hagit Miskin, Hannah Tamary
BACKGROUND: Most patients with anemia are diagnosed through clinical phenotype and basic laboratory testing. Nonetheless, in cases of rare congenital anemias, some patients remain undiagnosed despite undergoing an exhaustive workup. Genetic testing is complicated by the large number of genes involved in rare anemias and the similarities in the clinical presentation of the different syndromes. OBJECTIVE: We aimed to enhance the diagnosis of patients with congenital anemias by using targeted next-generation sequencing...
May 22, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29777631/effect-of-deferasirox-erythropoietin-vs-erythropoietin-on-erythroid-response-in-low-int-1-risk-mds-patients-results-of-the-phase-ii-kallisto-trial
#4
Norbert Gattermann, Rosa Coll, Lutz Jacobasch, Allameddine Allameddine, Amin Azmon, Laurie DeBonnett, Andreas Bruederle, Jie Jin
OBJECTIVES: Erythropoiesis-stimulating agents (ESAs) remain first-choice to treat symptomatic anemia and delay transfusion dependence in most patients with lower-risk myelodysplastic syndromes (MDS) without del(5q). Deferasirox increased erythroid responses in some lower-risk MDS patients in clinical trials, and adding low-dose deferasirox to ESA treatment may further improve erythroid response. METHODS: KALLISTO (NCT01868477) was a randomized, open-label, multicenter, phase II study...
May 19, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29763513/child-onset-thrombotic-thrombocytopenic-purpura-caused-by-p-r498c-and-p-g259pfsx133-mutations-in-adamts13
#5
An-Sofie Schelpe, Christelle Orlando, Bogaç Ercig, Chloë Geeroms, Inge Pareyn, Nele Vandeputte, Leydi Carolina Velásquez Pereira, Elien Roose, Karel Fostier, Gerry A F Nicolaes, Hans Deckmyn, Simon F De Meyer, Karen Vanhoorelbeke, Kristin Jochmans
INTRODUCTION: Patients suffering from congenital thrombotic thrombocytopenic purpura (cTTP) have a deficiency in ADAMTS13 due to mutations in their ADAMTS13 gene. OBJECTIVE: The aim of this study was to determine ADAMTS13 parameters (activity, antigen and mutations), to investigate if the propositus suffered from child-onset cTTP and to study the in vitro effect of the ADAMTS13 mutations. METHODS: ADAMTS13 activity and antigen were determined using the FRETS VWF73 assay and ELISA and ADAMTS13 mutations via sequencing of the exons...
May 15, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29758108/comparison-of-transplant-specific-prognostic-scoring-systems-in-haploidentical-transplantation-for-myelodysplastic-syndrome
#6
Seung-Hwan Shin, Young-Woo Jeon, Jae-Ho Yoon, Seung-Ah Yahng, Sung-Eun Lee, Byung-Sik Cho, Ki-Seong Eom, Seok Lee, Hee-Je Kim, Chang-Ki Min, Seok-Goo Cho, Dong-Wook Kim, Jong-Wook Lee, Woo-Sung Min, Yoo-Jin Kim
OBJECTIVES: We intended to identify the predictive abilities of recently published transplant-specific prognostic scoring systems in myelodysplastic syndrome (MDS) patients receiving haploidentical transplantation. METHODS: The outcomes of 73 MDS patients receiving haploidentical transplantation were analyzed, according to the MTPSS, the TRI, and the CIBMTR scoring systems. RESULTS: The median age of patients at transplantation was 50 (range, 19-69) years...
May 14, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29754401/treatment-of-localized-stage-follicular-lymphoma
#7
REVIEW
Marc Sorigue, Victòria Tuset, Juan-Manuel Sancho
Follicular lymphoma (FL) is the most common indolent lymphoma and it most frequently presents in an advanced-stage. Therapeutic considerations for advanced-stage are different from those of localized-stage FL, in which radiotherapy (RT) is generally recommended. However, the available evidence suffers from shortcomings that are relatively specific to this clinical entity due to its rarity and long survival with all available treatment modalities, including that most of the existing evidence originated at a time when diagnostic classifications, staging procedures and radiotherapeutic standards were different from those available today and when anti-CD20 monoclonal antibodies were not available...
May 12, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29742289/the-genetic-and-molecular-pathogenesis-of-myelodysplastic-syndromes
#8
REVIEW
Rory M Shallis, Rami Ahmad, Amer M Zeidan
Myelodysplastic syndromes (MDS) comprise a diverse group of clonal and malignant myeloid disorders characterized by ineffective hematopoiesis, resultant peripheral cytopenias, and a meaningful increased risk of progression to acute myeloid leukemia. A wide array of recurring genetic mutations involved in RNA splicing, histone manipulation, DNA methylation, transcription factors, kinase signaling, DNA repair, cohesin proteins, and other signal transduction elements have been identified as important substrates for the development of MDS...
May 9, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29729102/autoimmune-thrombocytopenic-purpura-among-patients-with-cancer-and-its-response-to-treatment
#9
Mahmoud H Ayesh Haj Yousef, Khaldoon Alawneh, Deeb Zahran, Najla H Aldaoud, Yousef Khader
OBJECTIVES: The aim of this study was to determine the rate of autoimmune thrombocytopenic purpura (ITP) in adult Jordanian patients diagnosed with cancer, to correlate this rate with the type of cancer, and to assess the response of ITP in cancer patients to treatment. METHODS: All adult patients aged 16 years or older who had been diagnosed with cancer at King Abdullah University Hospital (KAUH) between September 2002 and October 2017 were included in this study...
May 5, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29727497/greater-attention-should-be-paid-to-developing-therapies-for-elderly-patients-with-hodgkin-lymphoma-a-population-based-study-from-sweden
#10
Magnus Björkholm, Caroline E Weibull, Sandra Eloranta, Karin E Smedby, Ingrid Glimelius, Paul W Dickman
OBJECTIVE: Forty percent of Hodgkin lymphoma (HL) patients are older than 50 years at diagnosis, a fact which is not commonly recognized. Older patients do significantly worse than younger patients and are rarely included in clinical trials. METHODS: Using data from Swedish Cancer and Lymphoma Registries we estimated relative survival ratios (RSRs) for 7,997 HL patients (diagnosed 1973-2013; 45% ≥50 years). RESULTS: The 1-year RSRs (95% confidence interval; CI) for males aged 45-59, 60-69, 70-80, and 81 years and over, diagnosed in 2013, were 0...
May 4, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29719938/cyclophosphamide-s-addition-in-relapsed-refractory-multiple-myeloma-patients-with-biochemical-progression-during-lenalidomide-dexamethasone-treatment
#11
Laura Cesini, Agostina Siniscalchi, Sara Grammatico, Alessandro Andriani, Alessia Fiorini, Luca De Rosa, Tommaso Za, Angela Rago, Tommaso Caravita, Maria Teresa Petrucci
OBJECTIVE: The aim of this study was to evaluate the addition of Cyclophosphamide in relapsed-refractory Multiple Myeloma patients (RRMM) who experienced biochemical relapse or progression without CRAB, during treatment with Lenalidomide and Dexamethasone (Rd), to slow down the progression in active relapse. METHODS: This analysis included 31 patients with RRMM treated with Rd who received cyclophosphamide (CRd) at biochemical relapse. The CRd regimen was continued until disease progression...
May 2, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29719933/prospective-evaluation-of-the-effect-of-deferasirox-on-hematologic-response-in-transfusion-dependent-patients-with-low-risk-mds-and-iron-overload
#12
C Rose, C Lenoir, E Gyan, M Hacini, S Amé, B Corront, O Beyne-Rauzy, D Adiko, E Loppinet, N Ali-Ammar, K Laribi, E Wattel, F Dreyfus, C S Roué, S Cheze
OBJECTIVES: To assess the reduction of transfusions rate in transfusion-dependent patients with low-risk myelodysplastic syndrome (MDS) with iron overload treated with deferasirox. METHODS: Prospective observational study. Primary endpoint was reduction in transfusion requirements (RTR) at 3 months, (assessed on 8-week period). Secondary endpoints were hematologic improvement according IWG 2006 criteria at 3, 6, 12 months. RESULTS: Fifty-seven patients were evaluable...
May 2, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29719928/long-term-follow-up-of-2-patients-treated-with-90-y-rituximab-radioimmunotherapy-for-relapse-of-nodular-lymphocyte-predominant-hodgkin-lymphoma
#13
S Golstein, K Muylle, M Vercruyssen, C Spilleboudt, A de Wind, D Bron
Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare lymphoma (< 5% of Hodgkin's lymphomas) predominantly affecting the middle-aged man, with an indolent behavior. Given the rare occurrence of this lymphoma, there are currently no clear guidelines for initial treatment or relapse. In this report, we present the follow up of two patients treated by radioimmunotherapy for first relapse of their NLPHL. Both patients were initially treated with rituximab and relapsed 1 year after the end of their treatment...
May 2, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29719925/prognostic-markers-in-core-binding-factor-aml-and-improved-survival-with-multiple-consolidation-cycles-of-intermediate-high-dose-cytarabine
#14
Ashvind Prabahran, Mark Tacey, Shaun Fleming, Andrew Wei, Courtney Tate, Paula Marlton, Joel Wight, Andrew Grigg, Annabel Tuckfield, Jeff Szer, David Ritchie, Lynette Chee
OBJECTIVES: Core-binding factor acute myeloid leukaemia (CBF AML) defined by t(8;21)(q22;q22) or inv(16)(p13q22)/t(16;16)(p13;q22) has a favourable prognosis, however 30-40% of patients still relapse after chemotherapy. We sought to evaluate risk factors for relapse in a de novo CBF AML cohort. PATIENTS/MATERIALS/METHODS: A retrospective review of patients from 4 Australian tertiary centres from 2001-2012, comprising 40 t(8;21) and 30 inv(16) AMLs. RESULTS: Multivariate analysis identified age (p=0...
May 2, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29714032/alemtuzumab-versus-anti-thymocyte-globulin-in-patients-transplanted-from-an-unrelated-donor-after-a-reduced-intensity-conditioning
#15
Marie Robin, Kavita Raj, Sylvie Chevret, Jordan Gauthier, Hugues de Lavallade, David Michonneau, Donal McLornan, Régis Peffault de Latour, Victoria Potter, Austin Kulasekararaj, Flore Sicre de Fontbrune, Antonio Pagliuca, Ibrahim Yakoub-Agha, Gérard Socié, Ghulam J Mufti
OBJECTIVE: Relapse and graft-versus-host disease are still the main complications after allogeneic hematopoietic stem cell transplantation, especially in the setting of reduced intensity regimen (RIC) and unrelated donor. We compared here anti-thymocyte globulin (ATG) or alemtuzumab as GVHD prophylaxis in patients with myeloid disease transplanted after RIC and from an unrelated donor. METHOD: ATG- and alemtuzumab -patients have been matched by age, gender, HLA matching, comorbidities and cytogenetics risk (119 patients in each group)...
April 30, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29691899/droplet-digital-pcr-for-bcr-abl-p210-detecting-of-cml-a-high-sensitive-method-of-the-minimal-residual-disease-disease-progression
#16
Wen-Jun Wang, Chao-Feng Zheng, Zhuang Liu, Yan-Hong Tan, Xiu-Hua Chen, Bin-Liang Zhao, Guo-Xia Li, Zhi-Fang Xu, Fang-Gang Ren, Yao-Fang Zhang, Jian-Mei Chang, Hong-Wei Wang
OBJECTIVE: The present study intended to establish a droplet digital PCR (dd-PCR) for monitoring minimal residual disease (MRD) in patients with BCR/ABL (P210)-positive CML, thereby achieving deep-level monitoring of tumor load and determining the efficacy for guided clinically individualized treatment. METHODS: Using dd-PCR and RT-qPCR, two cell suspensions were obtained from K562 cells and normal peripheral blood mononuclear cells by gradient dilution and were measured at the cellular level...
April 25, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29676004/rapidly-changing-myeloma-epidemiology-in-the-general-population-increased-incidence-older-patients-and-longer-survival
#17
REVIEW
Ingemar Turesson, Magnus Bjorkholm, Cecilie Hveding Blimark, Sigurdur Kristinsson, Ramon Velez, Ola Landgren
The incidence of multiple myeloma is characterized by a steep increase with advancing age. Dramatic improvements in survival have been reported in clinical trials; however, elderly patients are generally underrepresented in these. The aims of this study are to review patterns of incidence and survival in multiple myeloma in the general population. We searched PubMed for population-based studies on trends in incidence and survival published between January 1, 2000 and June 30, 2017 and based on regional or national cancer registries and report the following results of the review...
April 20, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29663523/malignant-lymphoma-in-the-hiv-positive-patient
#18
REVIEW
Anne Meister, Marcus Hentrich, Christoph Wyen, Kai Hübel
The introduction of combination antiretroviral therapy (cART) drastically improved performance status, immune function and life expectancy of HIV-infected individuals. In addition, incidence of opportunistic infections and of AIDS-defining malignancies declined. Nevertheless, aggressive non-Hodgkin's lymphoma still remains the leading cause of AIDS-related deaths. The availability of cART, however, significantly improved the therapeutic options for HIV-positive patients with lymphomas. Diffuse large B-cell lymphoma, Burkitt's lymphoma, or Hodgkin lymphoma have increasingly become curable diseases...
April 16, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29660177/severe-adverse-events-by-tyrosine-kinase-inhibitors-decrease-survival-rates-in-patients-with-newly-diagnosed-chronic-phase-chronic-myeloid-leukemia
#19
Shuichi Ota, Toshihiro Matsukawa, Satoshi Yamamoto, Ito Shinichi, Motohiro Shindo, Kazuya Sato, Takeshi Kondo, Kyuhei Kohda, Hajime Sakai, Akio Mori, Tohru Takahashi, Hiroshi Ikeda, Hiroyuki Kuroda, Yoshihito Haseyama, Masaki Yamamoto, Takeo Sarashina, Makoto Yoshida, Ryoji Kobayashi, Mitsufumi Nishio, Toshimichi Ishihara, Yasuo Hirayama, Yasutaka Kakinoki, Hajime Kobayashi, Takashi Fukuhara, Masahiro Imamura, Mitsutoshi Kurosawa
OBJECTIVE: This multicenter cooperative study aimed to analyze the adverse events (AEs) associated with tyrosine kinase inhibitors (TKIs) used as initial treatment for chronic-phase chronic myeloid leukemia (CML-CP) and their impact on outcome. METHODS: We retrospectively evaluated 450 patients with CML-CP who received TKIs between 2004 and 2014. RESULTS: The 5-year overall survival (OS) and event-free survival (EFS) rates were 95.1% and 89...
April 16, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29660176/the-role-of-radiotherapy-as-salvage-and-or-consolidation-treatment-in-relapsed-refractory-and-high-risk-diffuse-large-b-cell-lymphoma
#20
Eric Grignano, Jérémy Laurent, Bénédicte Deau, Barbara Burroni, Didier Bouscary, Youlia M Kirova
OBJECTIVE: Many salvage therapies have been proposed for relapsed/refractory (R/R) diffuse large B-cell lymphomas or for consolidation in the case of suboptimal response. Radiotherapy (RT) is one modality of salvage therapy, but its place is currently not well defined. METHOD: This paper reports a retrospective review of patients receiving unplanned radiotherapy for R/R diffuse large B-cell lymphoma (DLBCL) or primary mediastinal B-cell lymphoma (PMBCL), or as consolidation therapy after second line chemotherapy, treated in our hospital...
April 16, 2018: European Journal of Haematology
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