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European Journal of Haematology

Marlijn Hoeks, Marit van der Pol, Rutger Middelburg, Dorothea Evers, Marian van Kraaij, Jaap Jan Zwaginga
OBJECTIVES: Secondary iron overload due to red blood cell transfusions (RBCT) is associated with increased morbidity and mortality. However, attention for secondary iron overload and its side effects in patients with hematological malignancies may need improvement. The aim of this study was to determine the number of transfused RBCT needed to reach a maximum bone marrow iron score (BMIS). METHODS: BMIS was independently assessed by two researchers on consecutive bone marrow samples of 35 acute myeloid leukemia (AML) patients...
July 17, 2018: European Journal of Haematology
Joanna Zawitkowska, Monika Lejman, Agnieszka Zaucha-Prażmo, Katarzyna Drabko, Marcin Płonowski, Joanna Bulsa, Michał Romiszewski, Agnieszka Mizia-Malarz, Andrzej Kołtan, Katarzyna Derwich, Grażyna Karolczyk, Tomasz Ociepa, Magdalena Ćwiklińska, Joanna Trelińska, Joanna Owoc-Lempach, Maciej Niedźwiecki, Aleksandra Kiermasz, Jerzy Kowalczyk
OBJECTIVE: The aim of this study was to analyse the clinical characteristics and outcome of children diagnosed with Ph+ ALL. MATERIAL AND METHODS: A total of 2591 newly diagnosed ALL children were treated in Poland between the years 2005-2017. Of those, 44 were diagnosed with Ph(+) ALL. The patients were treated according to protocols: ALL IC - BFM 2002 and 2009 (26 patients), EsPhALL (12 patients), initially ALL IC - BFM and then EsPhALL (6 patients). RESULTS: The median of follow up in the observed group was 3 years...
July 14, 2018: European Journal of Haematology
Anne Bouvier, Bénédicte Ribourtout, Sylvie François, Corentin Orvain, Damien Luque Paz, Annaëlle Beucher, Alexandre Guérard, Philippe Guardiola, Valérie Ugo, Odile Blanchet, Franck Geneviève, Aline Schmidt, Mathilde Hunault-Berger
Allogeneic hematopoietic stem cell transplantation (HSCT) is the one of treatment known to cure acute myeloid leukemia (AML). Relapse of AML remains the major cause of treatment failure in patients after HSCT. In rare cases, secondary leukemia is derived from donor cells, designated as donor cell leukemia (DCL) This article is protected by copyright. All rights reserved.
July 14, 2018: European Journal of Haematology
Hung Chang, Tzung-Chih Tang, Yu-Shin Hung, Pei-Ling Li, Ming-Chung Kuo, Jin-Hou Wu, Po-Nan Wang
OBJECTIVE: For immune thrombocytopenia (ITP), efficacy of frontline steroids is well established. However, clinical data comparing various treatment options for refractory or relapsed ITP are limited. We aimed to investigate the outcome of frontline steroid treatment for ITP patients and compare common second line modalities in a single institute in Taiwan. METHODS: We collected the complete outpatient list over a 6-month period. Patients were identified from the list and medical records were reviewed to capture the data retrospectively...
July 14, 2018: European Journal of Haematology
Hanne E H Møller, Birgitte S Preiss, Per Pedersen, Brian Østergaard, Mikael Frederiksen, Niels Abildgaard, Michael B Møller
OBJECTIVE: Prognostic and predictive markers in multiple myeloma are continuously explored because of the heterogeneity of the tumor biology. Myc protein is the final product from activating MYC oncogene but the prognostic impact in multiple myeloma is not well described. METHODS: In a population-based cohort of 194 untreated, newly diagnosed patients with multiple myeloma, we assessed myc protein expression using CD138/myc immunohistochemical double stain and collected clinicopathological data...
July 12, 2018: European Journal of Haematology
Sherif M Badawy, Amanda B Payne, Mark J Rodeghier, Robert I Liem
OBJECTIVES: To determine factors associated with exercise capacity in adults with sickle cell disease (SCD) and its relationship to hospitalizations and mortality. METHODS: A total of 223 participants in the Cooperative Study of Sickle Cell Disease (CSSCD) (64% female, 70% hemoglobin SS/Sβ0 thalassemia, mean age 43.3 ± 7.5 years) underwent maximal exercise testing using a treadmill protocol with a mean duration of 11.6 ± 5.2 minutes. RESULTS: Female sex (β = -3...
July 12, 2018: European Journal of Haematology
Ada H V Repetto-Llamazares, Marion M Malenge, Adam O'Shea, Bergthóra Eiríksdóttir, Trond Stokke, Roy H Larsen, Jostein Dahle
OBJECTIVES: To investigate the therapeutic potential of the next generation anti-CD37 radioimmunoconjugate 177 Lu-lilotomab satetraxetan (177 Lu-lilotomab) in combination with the anti-CD20 antibody rituximab for treatment of mice with non-Hodgkin's lymphoma (NHL) xenografts. METHODS: Nude mice with subcutaneous (s.c.) Burkitt's lymphoma Daudi xenografts and SCID mice intravenously (i.v.) injected with Mantle cell lymphoma Rec-1 cells were treated with either 177 Lu-lilotomab or rituximab alone or with the combination of both treatments...
July 11, 2018: European Journal of Haematology
Eva U Spukti, Lars Henning Schmidt, Arik Schulze, Christoph Schliemann, Dennis Görlich, Eva Wardelmann, Wolfgang Hartmann, Georg Lenz, Wolfgang E Berdel, Andrea Kerkhoff
BACKGROUND: Follicular lymphoma (FL) is the most frequent indolent lymphoma subtype in adults. Maintenance therapy with rituximab is frequently applied to FL patients with complete or partial response following initial chemoimmunotherapy. However, radioimmunotherapy with 90 Y-ibritumomab-tiuxetan represents a therapeutic alternative. METHODS: To compare the clinical and the prognostic impact of both therapies, a study collective of n = 56 patients diagnosed with indolent B-cell lymphoma was retrospectively investigated...
July 11, 2018: European Journal of Haematology
Hanna Karvonen, Robert Perttilä, Wilhelmiina Niininen, Harlan Barker, Daniela Ungureanu
Recent studies showed that several pseudokinases from the receptor tyrosine kinase family are important players in regulating cancer cell invasion, metastasis and drug-resistance, suggesting that targeting these proteins can play a therapeutic role in cancer treatment. Receptor Tyr kinase-like orphan receptors (RORs), protein Tyr kinase 7 (PTK7) (also called colon carcinoma kinase 4 (CCK4)), and receptor-like Tyr kinase (RYK) are Wnt ligand binding receptors within the non-canonical Wnt signaling, with important roles in development, tissue homeostasis and organogenesis...
July 10, 2018: European Journal of Haematology
Yoshinori Hashimoto, Hirohisa Nakamae, Takayuki Tanaka, Hiromi Omura, Mirei Horiuchi, Takuro Yoshimura, Teruhito Takakuwa, Atsuko Mugitani, Asao Hirose, Mika Nakamae, Hideo Koh, Masayuki Hino
OBJECTIVE: We examined the prognostic factors to validate previous prognostic models for survival and thrombosis with large-scale data on Japanese patients with essential thrombocythemia (ET). METHOD: We conducted a study in 352 patients with ET to validate previous prognostic models and search for new prognostic factors. RESULTS: The International Prognostic Score for essential thrombocythemia (IPSET), the conventional risk classification, and the International Prognostic Score for thrombosis in essential thrombocythemia (IPSET-T) were confirmed to be reproducible in Japanese patients...
July 4, 2018: European Journal of Haematology
Corentin Orvain, Lydie Da Costa, Richard Van Wijk, Serge Pissard, Véronique Picard, Lamisse Mansour-Hendili, Séverine Cunat, Muriel Giansily-Blaizot, Guillaume Cartron, Jean-François Schved, Patricia Aguilar-Martinez
Severe iron overload is frequent in dehydrated hereditary stomatocytosis (DHSt) despite well compensated hemolysis and no or little transfusion requirement. We investigated 4 patients with proven DHSt, in whom the degree of hemolysis was closely related with iron status. Genetic modifiers increasing iron stores (HFE:pCys282Tyr, HAMP:c-153C>T mutations) were accompanied with high liver iron concentrations and increased hemolysis whereas therapeutic phlebotomies alleviated the hemolytic phenotype. There were no manifestations of hemolysis in one patient with low iron stores...
July 3, 2018: European Journal of Haematology
Vered Yahalom, Nir Pillar, Yingying Zhao, Shirley Modan, Mingyan Fang, Lydia Yosephi, Orna Asher, Eilat Shinar, Gershon Celniker, Haike Resnik-Wolf, Yael Brantz, Hagit Hauschner, Nurit Rosenberg, Le Cheng, Noam Shomron, Elon Pras
BACKGROUND: AnWj is a high-incidence blood group antigen associated with 3 clinical disorders: lymphoid malignancies, immunologic disorders, and autoimmune hemolytic anemia. The aim of this study was to determine the genetic basis of an inherited AnWj negative phenotype. METHODS: We identified a consanguineous family with two AnWj negative siblings and 4 additional AnWj negative individuals without known familial relationship to the index family. We performed exome sequencing in search for rare homozygous variants shared by the two AnWj negative siblings of the index family and searched for these variants in the four non-related AnWj negative individuals...
June 29, 2018: European Journal of Haematology
Uri Greenbaum, Erel Joffe, Kalman Filanovsky, Howard S Oster, Ilya Kirgner, Itai Levi, Pia Raanani, Irit Avivi, Esther Manor, Gili Man-El, Moshe Mittelman
OBJECTIVES: To ascertain the relevance of bone marrow cellularity (BMC) to the interpretation of blast percentage (blast%) in MDS prognostication. METHODS: We compared survival prediction based on blast% adjusted to different levels of cellularity, compared to the survival based on the original IPSS-R blast% grouping. RESULTS: We analyzed 355 consecutive MDS patient. Cellularity, in and of itself or its interaction with blast%, were not associated with overall survival (OS)...
June 29, 2018: European Journal of Haematology
Anna Bosch-Vilaseca, Irene García-Cadenas, Elisa Roldán, Silvana Novelli, Pere Barba, Albert Esquirol, David Valcárcel, Rodrigo Martino, Jorge Sierra
OBJECTIVE AND METHODS: Severe post-engraftment thrombocytopenia is a common complication after allogeneic stem cell transplantation (alloSCT). A few studies have suggested that the use of thrombopoietin agonists (TPOa) may be useful in this setting. Our retrospective study is the largest series published to date; we retrospectively evaluated TPOa efficacy and safety in 20 adult alloSCT recipients who received TPOa as a compassionate use for clinically-relevant thrombocytopenia. RESULTS: Twelve of 20 patients (60%) responded, with a 180-day cumulative incidence of successful platelet recovery to ≥30 and ≥50 x 109 /L of 57% (95% C...
June 29, 2018: European Journal of Haematology
Yuhua Ru, Jia Chen, Depei Wu
EBV viremia and PTLD are severe complications after hematopoietic stem cell transplantation (HSCT). A series of risk factors have been found to predict EBV viremia and PTLD, including the T-cell depletion, reduced intensity conditioning and alternative donor, etc. The rituximab pre-emptive therapy could improve PTLD prognosis significantly, but the trigger of initiating rituximab pre-emptive therapy has not been well established. Additionally, EBV specific cytotoxic T-cell (CTL) is a promising approach to treat EBV-PTLD...
June 27, 2018: European Journal of Haematology
Salah Abohelaika, Hilary Wynne, Peter Avery, Brian Robinson, Lisa Jones, Campbell Tait, Bradley Dickinson, Julie Salisbury, Joanna Nightingale, Louise Green, Farhad Kamali
OBJECTIVES: Time within therapeutic INR range (TTR) predicts benefits/risk of warfarin therapy. Identification of individual- and centre-related factors that influence TTR, and addressing them to improve anticoagulation control, are important. This study examined the impact of individual and centre-related factors upon long-term anticoagulation control in atrial fibrillation patients in seven UK-based monitoring services. METHODS: Data between 2000 and 2014 on 25,270 patients (equating to 203,220 patient years) [18,120 (71...
June 27, 2018: European Journal of Haematology
Erel Joffe, Neta Goldschmidt, Osnat Bairey, Riva Fineman, Rosa Ruchlemer, Naomi Rahimi-Levene, Lev Shvidel, Uri Greenbaum, Ariel Aviv, Tamar Tadmor, Andrea Braester, Ariela Arad, Aaron Polliack, Yair Herishanu
OBJECTIVE: To evaluate disease characteristics and long-term outcomes in patients requiring second-line treatment following FCR, for relapsed/refractory disease (R/R), or following discontinuation due to toxicities. METHOD: A retrospective analysis of 126 CLL patients treated with frontline FCR: 63 received second-line treatment (41 relapsed, 9 refractory (SD/PD), 13 prior toxicity). Time to next treatment (TTNT) was calculated from beginning FCR to initiation of second-line therapy...
June 27, 2018: European Journal of Haematology
Rachael F Grace, Jennifer Cohen, Shayna Egan, Ted Wells, Brooke Witherspoon, Aisling Ryan, Sam S Salek, Susan Bodie, Robert J Klaassen
OBJECTIVES: This study explored how signs and symptoms of pyruvate kinase (PK) deficiency, a rare hemolytic anemia caused by mutations in the PKLR gene, impacts patients' health-related quality of life (HRQoL). METHODS: Interviews with twenty-one adults with PK deficiency in the United States, Netherlands, and Germany were conducted. Participants were asked to describe signs, symptoms, and impacts of the disease on their daily lives. Interviews were transcribed and analyzed using qualitative analysis methods...
June 23, 2018: European Journal of Haematology
Kundan Mishra, Aditya Jandial, Deepesh Lad, Gaurav Prakash, Alka Khadwal, Neelam Varma, Pankaj Malhotra
We read with keen interest the article written by Krista Vaht et al. (1) about the low response rate to ATG-based immunotherapy in very severe aplastic anemia. The article nicely describes the real world data of response rate of ATG-based immunosuppressive therapy in very severe aplastic anemia patients. However, we would like to add two more points, cost and complications (infectious) of ATG, to the article, which are extremely important in the resource constraints setting of developing countries. This article is protected by copyright...
June 19, 2018: European Journal of Haematology
Joslyn Rudoni, Anna Jan, Chitra Hosing, Fleur Aung, Jason Yeh
OBJECTIVE: To evaluate response rates and survival in adults with transplant-associated thrombotic microangiopathy (TA-TMA) after allogeneic hematopoietic stem cell transplantation (HSCT) who were treated with eculizumab (ECU). METHODS: Patients were identified retrospectively and data collected through HSCT and pharmacy databases. RESULTS: Ten patients with TA-TMA after allogeneic HSCT were treated with ECU between 2013 and 2016. TA-TMA was diagnosed at a median of 93 days post-HSCT...
June 19, 2018: European Journal of Haematology
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