Read by QxMD icon Read


Immacolata Andolfo, Roberta Russo, Antonella Gambale, Achille Iolascon
After the first proposed model of the red blood cell membrane skeleton 36 years ago, several additional proteins have been discovered during the overriding years, and their relationship with the pathogenesis of the related disorders have been somewhat defined. The knowledge of erythrocyte membrane structure is important because it represents the model for spectrin-based membrane skeletons in all cells and because defects in its structure underlie multiple hemolytic anemias. This review summarized the main features of erythrocyte membrane disorders, dividing them in structural and altered permeability defects, particularly focusing on the most recent advances...
October 18, 2016: Haematologica
(no author information available yet)
In 2014, ibrutinib was made available for relapsed/refractory chronic lymphocytic leukaemia (CLL) patients. The UK CLL Forum collected data from UK/Ireland patients with a minimum of 1 year follow-up with pre-planned primary endpoints; the number of patients still on therapy at 1 year (Discontinuation Free Survival; DFS) and 1 year overall survival (OS). With a median 16 months follow-up, data on 315 patients demonstrated 1 year DFS of 73.7% and 1 year OS of 83.8%. Patients with better pre-treatment performance status (PS 0/1 vs 2+) had superior DFS (77...
October 18, 2016: Haematologica
Áine M Prendergast, Andrea Kuck, Mieke van Essen, Simon Haas, Sandra Blaszkiewicz, Marieke A G Essers
In the bone marrow, endothelial cells are a major component of the hematopoietic stem cell vascular niche and are a first line of defense against inflammatory stress and infection. The primary response of an organism to infection involves the synthesis of immune-modulatory cytokines, including interferon alpha. In the bone marrow, interferon alpha induces rapid cell cycle entry of hematopoietic stem cells in vivo However, the effect of interferon alpha on bone marrow endothelial cells has not been described...
October 14, 2016: Haematologica
Frédéric Courtier, Nadine Carbuccia, Séverine Garnier, Arnaud Guille, José Adelaïde, Nathalie Cervera, Veronique Gelsi-Boyer, Marie-Joelle Mozziconacci, Jérôme Rey, Nobert Vey, Max Chaffanet, Daniel Birnbaum, Anne Murati
No abstract text is available yet for this article.
October 14, 2016: Haematologica
Rohit Mathur, Lalit Sehgal, Ondrej Havranek, Stefan Köhrer, Tamer Khashab, Neeraj Jain, Jan A Burger, Sattva S Neelapu, R Eric Davis, Felipe Samaniego
Histone methylation and demethylation regulates B-cell development, and their deregulation correlates with tumor chemoresistance in diffuse large B-cell lymphoma, limiting curative rates. Since histone methylation status correlates with disease aggressiveness and relapse, we investigated the therapeutic potential of inhibiting histone 3 Lys27 demethylase KDM6B, in-vitro, using the small molecule inhibitor GSK-J4. KDM6B is overexpressed in the DLBCL germinal center B-cell subtype, and higher KDM6B levels are associated with worse survival in DLBCL patients treated with R-CHOP...
October 14, 2016: Haematologica
Mohib Uddin, Catherine Betts, Ian Robinson, Anna Malmgren, Charles Humfrey
No abstract text is available yet for this article.
October 14, 2016: Haematologica
Marina Kunstreich, Sebastian Kummer, Hans-Juergen Laws, Arndt Borkhardt, Michaela Kuhlen
The morbidity and toxicity associated with current intensive treatment protocols for acute lymphoblastic leukemia in childhood become even more important as the vast majority of children can be cured and become long-term survivors. Osteonecrosis is one of the most common therapy-related and debilitating side effects of anti-leukemic treatment and can adversely affect long-term quality of life. Incidence and risk factors vary substantially between study groups and therapeutic regimens. We therefore analyzed 22 clinical trials of childhood acute lymphoblastic leukemia in terms of osteonecrosis incidence and risk factors...
October 14, 2016: Haematologica
Marialaura Bonaccio, Augusto Di Castelnuovo, George Pounis, Amalia De Curtis, Simona Costanzo, Mariarosaria Persichillo, Chiara Cerletti, Maria Benedetta Donati, Giovanni de Gaetano, Licia Iacoviello
Low-grade inflammation is associated with increased risk of chronic degenerative disease but its relationship with mortality is less explored. We aimed at evaluating, at a large epidemiological level, the possible association of low-grade inflammation, as measured by a composite score, with overall mortality risk. We conducted a population-based prospective investigation on 20,337 adult subjects free from major haematological disease and acute inflammatory status, randomly recruited from the general population of the Moli-sani study...
October 14, 2016: Haematologica
Aurélie Chauveau, Olivier Nibourel, Sylvie Tondeur, Damien Luque Paz, Olivier Mansier, Franciane Paul, Mathieu Wemeau, Claude Preudhomme, Eric Lippert, Valérie Ugo
No abstract text is available yet for this article.
October 6, 2016: Haematologica
Haiwei Lian, Dun Li, Yun Zhou, Esther Landesman-Bollag, Guanglan Zhang, Nicole M Anderson, Kevin Charles Tang, Justine E Roderick, Michelle A Kelliher, David C Seldin, Hui Fu, Hui Feng
No abstract text is available yet for this article.
October 6, 2016: Haematologica
Kevin Rouault-Pierre, Alexander E Smith, Syed A Mian, Irene Pizzitola, Austin G Kulasekararaj, Ghulam J Mufti, Dominique Bonnet
No abstract text is available yet for this article.
October 6, 2016: Haematologica
Clémentine Sarkozy, Thierry Molina, Hervé Ghesquières, Anne-Sophie Michallet, Jehan Dupuis, Diane Damotte, Franck Morsschauser, Marie Parrens, Laurent Martin, Peggy Dartigues, Aspasia Stamatoullas, Pierre Hirsch, Betina Fabiani, Krimo Bouabdallah, Maria Gomes da Silva, Marie Maerevoet, Camille Laurent, Bertrand Coiffier, Gilles Salles, Alexandra Traverse-Glehen
Mediastinal grey zone lymphoma, B cell lymphomas with intermediate features between classical Hodgkin lymphoma and primary mediastinal B cell lymphoma, are not well described in the literature. We report the clinical characteristics and outcomes of a large retrospective series of 99 cases centrally reviewed by a panel of hematopathologists, with a consensus established for the diagnosis. Cases were defined as classical Hodgkin lymphoma-like morphology (64.6%) with primary mediastinal B cell lymphoma immunophenotype, primary mediastinal B cell lymphoma-like morphology (30...
October 6, 2016: Haematologica
Annalisa Ruggeri, Yuqian Sun, Myriam Labopin, Andrea Bacigalupo, Francesca Lorentino, William Arcese, Stella Santarone, Zafar Gülbas, Didier Blaise, Giuseppe Messina, Ardeshi Ghavamzadeh, Florent Malard, Benedetto Bruno, Jose Luis Diez-Martin, Yener Koc, Fabio Ciceri, Mohamad Mohty, Arnon Nagler
Severe graft-versus-host-disease is a major barrier for non T-cell-depleted haploidentical stem cell transplantation and there is no consensus on the optimal GVHD prophylaxis. This study compared the two most commonly used graft-versus-host-disease prophylaxis regimens (post-transplant-cyclophosphamide-based (PTCY) versus the anti-thymocyte-globulin-based (ATG)) in adults with acute myeloid leukemia reported to the european society for blood and bone marrow transplantation. 308 patients were analyzed, 193 received PTCY and 115 ATG as anti- graft-versus-host-disease prophylaxis...
October 6, 2016: Haematologica
Kyungho Kim, Jing Li, Andrew Barazia, Alan Tseng, Seock-Won Youn, Giovanni Abbadessa, Yi Yu, Brian Schwartz, Robert K Andrews, Victor R Gordeuk, Jaehyung Cho
Previous studies identified the Ser/Thr protein kinase, AKT, as a therapeutic target for thromboinflammatory diseases. Here we report that specific inhibition of AKT with ARQ 092, an orally-available AKT inhibitor currently in Phase Ib clinical trials as an anti-cancer drug, attenuates the adhesive function of neutrophils and platelets from sickle cell disease patients in vitro and cell-cell interactions in a mouse model of sickle cell disease. Studies using neutrophils and platelets isolated from sickle cell disease patients revealed that treatment with 50-500 nM ARQ 092 significantly blocks αMβ2 integrin function in neutrophils and reduces P-selectin exposure and glycoprotein Ib/IX/V-mediated agglutination in platelets...
October 6, 2016: Haematologica
Bagirath Gangadharan, Mathieu Ing, Sandrine Delignat, Ivan Peyron, Maud Teyssandier, Srinivas V Kaveri, Sébastien Lacroix-Desmazes
The development of inhibitory antibodies to therapeutic FVIII is the major complication of replacement therapy in patients with hemophilia A. The first step in the initiation of the anti-FVIII immune response is FVIII interaction with receptor(s) on antigen-presenting cells followed by endocytosis and presentation to naive CD4+ T cells. Recent studies indicate a role for the C1 domain in FVIII uptake. We investigated whether charged residues in the C2 domain participate in immunogenic FVIII uptake. Co-incubation of FVIII with BO2C11, a monoclonal C2-specific IgG, reduced FVIII endocytosis by dendritic cells and presentation to CD4+ T cells, and diminished FVIII immunogenicity in FVIII-deficient mice...
October 6, 2016: Haematologica
Theresia M Westers, Eline M P Cremers, Uta Oelschlaegel, Ulrika Johansson, Peter Bettelheim, Sergio Matarraz, Alberto Orfao, Bijan Moshaver, Lisa Eidenschink Brodersen, Michael R Loken, Denise A Wells, Dolores Subira', Matthew Cullen, Jeroen G Te Marvelde, Vincent H J Van der Velden, Frank W M B Preijers, Sung-Chao Chu, Jean Feuillard, Estelle Guérin, Katherina Psarra, Anna Porwit, Leonie Saft, Robin Ireland, Timothy Milne, Marie C Béné, Birgit I Witte, Matteo G Della Porta, Wolfgang Kern, Arjan A van de Loosdrecht
Current recommendations for diagnosing myelodysplastic syndromes endorse flow cytometry as an informative tool. Most flow cytometry protocols focus on the analysis of progenitor cells and the evaluation of the maturing myelomonocytic lineage. However, one of the most frequently observed features of myelodysplastic syndromes is anemia, which may be associated with dyserythropoiesis. Therefore, analysis of changes in flow cytometry features of nucleated erythroid cells may complement current flow cytometry tools...
October 6, 2016: Haematologica
Monique C Minnema, Eva Kimby, Shirley D'Sa, Luc-Matthieu Fornecker, Stéphanie Poulain, Tom J Snijders, Efstathios Kastritis, Stéphane Kremer, Aikaterini Fitsiori, Laurence Simon, Frédéric Davi, Michael Lunn, Jorge J Castillo, Christopher J Patterson, Magali Le Garff-Tavernier, Myrto Costopoulos, Véronique Leblond, Marie-José Kersten, Meletios A Dimopoulos, Steven P Treon
Bing Neel syndrome is a rare disease manifestation of Waldenstrom macroglobulinemia that results from infiltration of the central nervous system by malignant lymphoplasmacytic cells. In this guideline, we describe the clinical symptoms, as well as the appropriate laboratory and radiological studies that can aid in the diagnosis. The presentation of Bing Neel syndrome may be very diverse, and include headaches, cognitive deficits, paresis, and psychiatric symptoms. The syndrome can present in patients with known Waldenstrom macroglobulinemia, even in the absence of systemic progression, but also in previously undiagnosed patients...
October 6, 2016: Haematologica
Uri Rozovski, Srdan Verstovsek, Taghi Manshouri, Vilma Dembitz, Ksenija Bozinovic, Kate Newberry, Ying Zhang, Joseph E Bove, Sherry Pierce, Hagop Kantarjian, Zeev Estrov
In most patients with primary myelofibrosis, one of three mutually exclusive somatic mutations is detected. In approximately 60% of patients, the Janus kinase 2 gene is mutated; in 20%, the calreticulin gene is mutated; and in 5%, the myeloproliferative leukemia virus gene is mutated. Although patients with mutated calreticulin or myeloproliferative leukemia have a favorable outcome and those with none of these mutations have an unfavorable outcome, prognostication based on mutation status is challenging owing to the heterogeneous survival of patients with mutated Janus kinase 2...
September 29, 2016: Haematologica
Alberto Alvarez-Larrán, Manuel Pérez-Encinas, Francisca Ferrer-Marin, Juan-Carlos Hernández-Boluda, María José Ramírez, Joaquín Martínez-López, Elena Magro, Yasmina Cruz, María Isabel Mata, Pilar Aragües, María Laura Fox, Beatriz Cuevas, Sara Montesdeoca, José Angel Hernández-Rivas, Valentin García-Gutiérrez, María Teresa Gómez-Casares, Juan Luis Steegmann, María Antonia Durán, Montse Gómez, Ana Kerguelen, Abelardo Bárez, Mari Carmen García, Concepción Boqué, José María Raya, Clara Martinez, Manuel Albors, Francesc Garcia, Carmen Burgaleta, Carlos Besses
Hematocrit control below 45% is associated with a lower rate of thrombosis in polycythemia vera. In patients receiving hydroxyurea, this target can be achieved with hydroxyurea alone or with the combination of hydroxyurea plus phlebotomies. However, the clinical implications of phlebotomy requirement under hydroxyurea therapy are unknown. For such purpose, need of additional phlebotomies during the first 5 years of hydroxyurea therapy was evaluated in 533 patients with polycythemia vera. Patients requiring 3 or more phlebotomies per year (n=85, 16%) showed a worse hematocrit control than those requiring 2 or less phlebotomies per year (n=448, 84%)...
September 29, 2016: Haematologica
Sebastian Giebel, Myriam Labopin, Gerard Socie', Dietrich Beelen, Paul Browne, Liisa Volin, Slawomira Kyrcz-Krzemien, Ibrahim Yakoub-Agha, Mahmoud Aljurf, Depei Wu, Mauricette Michallet, Renate Arnold, Mohamad Mohty, Arnon Nagler
Allogeneic hematopoietic cell transplantation is widely used to treat adults with high risk acute lymphoblastic leukemia. The aim of this study was to analyze whether the results changed over time and to identify prognostic factors. Adult patients treated between year 1993 and 2012 with myeloablative allogeneic hematopoietic cell transplantation from HLA matched sibling (n=2681) or unrelated donors (n=2178) in first complete remission were included. For transplantations from sibling donors performed between 2008-2012, the following two-year probabilities of the overall survival were obtained: 76% (18-25 years old group), 69% (26-35 and 36-45 years old groups) and 60% (46-55 years old group)...
September 29, 2016: Haematologica
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"