journal
MENU ▼
Read by QxMD icon Read
search

Haematologica

journal
https://www.readbyqxmd.com/read/28302714/ruxolitinib-a-potent-jak1-jak2-inhibitor-induces-temporary-reductions-in-the-allelic-burden-of-concurrent-csf3r-mutations-in-chronic-neutrophilic-leukemia
#1
Arief S Gunawan, Donal P McLornan, Bridget Wilkins, Katherine Waghorn, Yvette Hoade, Nick C P Cross, Claire N Harrison
No abstract text is available yet for this article.
March 16, 2017: Haematologica
https://www.readbyqxmd.com/read/28302713/family-cord-blood-banking-for-sickle-cell-disease-a-twenty-year-experience-in-two-dedicated-public-cord-blood-banks
#2
Hanadi Rafii, Françoise Bernaudin, Helene Rouard, Valérie Vanneaux, Annalisa Ruggeri, Marina Cavazzana, Valerie Gauthereau, Aurélie Stanislas, Malika Benkerrou, Mariane De Montalembert, Christele Ferry, Robert Girot, Cecile Arnaud, Annie Kamdem, Joelle Gour, Claudine Touboul, Audrey Cras, Mathieu Kuentz, Claire Rieux, Fernanda Volt, Barbara Cappelli, Karina T Maio, Annalisa Paviglianiti, Chantal Kenzey, Jerome Larghero, Eliane Gluckman
Efforts to implement family cord blood banking have been developed in the past decades for siblings requiring stem cell transplantation for conditions such as sickle cell disease. However, public banks are faced with challenging decisions about the units to be stored, discarded, or used for other endeavors. We report here 20 years of experience in family cord blood banking for sickle cell disease in two dedicated public banks. Participants were pregnant women who had previous child diagnosed with homozygous sickle cell disease...
March 16, 2017: Haematologica
https://www.readbyqxmd.com/read/28302712/improved-survival-after-acute-graft-vs-host-disease-diagnosis-in-the-modern-era
#3
Hanna J Khoury, Tao Wang, Michael T Hemmer, Daniel Couriel, Amin Alousi, Corey Cutler, Mahmoud Aljurf, Joseph H Antin, Mouhab Ayas, Minoo Battiwalla, Jean-Yves Cahn, Mitchell Cairo, Yi-Bin Chen, Robert Peter Gale, Shahrukh Hashmi, Robert J Hayashi, Madan Jagasia, Mark Juckett, Rammurti T Kamble, Mohamed Kharfan-Dabaja, Mark Litzow, Navneet Majhail, Alan Miller, Taiga Nishihori, Muna Qayed, Helene Schoemans, Harry C Schouten, Gérard Socié, Jan Storek, Leo Verdonck, Ravi Vij, William A Wood, Lolie Yu, Rodrigo Martino, Matthew Carabasi, Christopher Dandoy, Usama Gergis, Peiman Hematti, Melham Solh, Kareem Jamani, Leslie Lehmann, Bipin Savani, Kirk R Schultz, Baldeep M Wirk, Stephen Spellman, Mukta Arora, Joseph Pidala
Acute graft vs. host disease remains a major threat to successful outcome after allogeneic hematopoietic cell transplantation. While improvements in treatment and supportive care have occurred, it is unknown whether these advances have resulted in improved outcome specifically among those diagnosed with acute graft vs. host disease. We examined outcome following diagnosis of grade II-IV acute graft vs. host disease according to time period, and examine effects according to original graft vs. host disease prophylaxis regimen and maximum overall grade of acute GVHD...
March 16, 2017: Haematologica
https://www.readbyqxmd.com/read/28302711/precision-and-prognostic-value-of-clone-specific-minimal-residual-disease-in-acute-myeloid-leukemia
#4
Pierre Hirsch, Ruoping Tang, Nassera Abermil, Pascale Flandrin, Hannah Moatti, Fabrizia Favale, Ludovic Suner, Florence Lorre, Christophe Marzac, Fanny Fava, Anne-Claire Mamez, Simona Lapusan, Françoise Isnard, Mohamad Mohty, Ollivier Legrand, Luc Douay, Chrystele Bilhou-Nabera, François Delhommeau
The genetic landscape of adult acute myeloid leukemias has been recently unraveled. However, due to their genetic heterogeneity, only a handful of markers are currently used for the evaluation of minimal residual disease. Recent studies using multi-target strategies indicate that detection of residual mutations in less than 5% of cells in complete remission is associated with a better survival. Here, in a series of 69 acute myeloid leukemias with known clonal architecture, we design a clone-specific strategy based on fluorescent in situ hybridization and high-sensitivity next generation sequencing to detect chromosomal aberrations and mutations, respectively, in follow-up samples...
March 16, 2017: Haematologica
https://www.readbyqxmd.com/read/28280079/targeted-therapy-for-a-subset-of-acute-myeloid-leukemias-that-lack-expression-of-aldehyde-dehydrogenase-1a1
#5
Maura Gasparetto, Shanshan Pei, Mohammad Minhajuddin, Nabilah Khan, Daniel A Pollyea, Jason R Myers, John M Ashton, Michael W Becker, Vasilis Vasiliou, Keith R Humphries, Craig T Jordan, Clayton A Smith
Aldehyde dehydrogenase 1A1 (ALDH1A1) activity is high in hematopoietic stem cells and functions in part to protect stem cells from reactive aldehydes and other toxic compounds. In contrast, we found that ~25% of all acute myeloid leukemias expressed low or undetectable levels of ALDH1A1 and that this ALDH1A1- subset of leukemias correlates with good prognosis cytogenetics. ALDH1A1- cell lines as well as primary leukemia cells were found to be sensitive to treatment with compounds that directly and indirectly generate toxic ALDH substrates including 4-hydroxynonenal and the clinically relevant compounds arsenic trioxide and 4-hydroperoxycyclophosphamide...
March 9, 2017: Haematologica
https://www.readbyqxmd.com/read/28280078/pathophysiological-consequences-and-benefits-of-hfe-mutations-20-years-of-research
#6
Ina Hollerer, André Bachmann, Martina U Muckenthaler
Mutations in the HFE (hemochromatosis) gene cause hereditary hemochromatosis, an iron overload disorder that is hallmarked by excessive accumulation of iron in parenchymal organs. The HFE mutation p.Cys282Tyr is pathologically most relevant and occurs in the Caucasian population with a carrier frequency of up to 1 in 8 in specific European regions. Despite this high prevalence, the mutation causes a clinically relevant phenotype only in a minority of cases. In this review, we summarize historical facts and recent research findings about hereditary hemochromatosis and outline the pathological consequences of the associated gene defects...
March 9, 2017: Haematologica
https://www.readbyqxmd.com/read/28280077/survival-of-patients-with-lymphoplasmacytic-lymphoma-and-solitary-plasmacytoma-in-germany-and-the-united-states-in-the-early-21st-century
#7
Janick Weberpals, Dianne Pulte, Lina Jansen, Sabine Luttmann, Bernd Holleczek, Alice Nennecke, Meike Ressing, Alexander Katalinic, Maximilian Merz, Hermann Brenner
No abstract text is available yet for this article.
March 9, 2017: Haematologica
https://www.readbyqxmd.com/read/28255023/the-clinical-and-molecular-diversity-of-mast-cell-leukemia-with-or-without-associated-hematologic-neoplasm
#8
Mohamad Jawhar, Juliana Schwaab, Manja Meggendorfer, Nicole Naumann, Hans-Peter Horny, Karl Sotlar, Torsten Haferlach, Karla Schmitt, Alice Fabarius, Peter Valent, Wolf-Karsten Hofmann, Nicholas C P Cross, Georgia Metzgeroth, Andreas Reiter
Mast cell leukemia is a rare variant of advanced systemic mastocytosis characterized by ≥20% mast cells in a bone marrow smear. We evaluated clinical and molecular characteristics of 28 patients with (n=20, 71%) or without an associated hematologic neoplasm. De novo mast cell leukemia was diagnosed in 16/28 (57%) patients and secondary mast cell leukemia evolving from other advanced systemic mastocytosis subtypes in 12/28 (43%) patients, of which 7 patients progressed while on cytoreductive treatment. Median bone marrow mast cell infiltration was 65% and median serum tryptase was 565 microg/L...
March 2, 2017: Haematologica
https://www.readbyqxmd.com/read/28255022/molecular-features-of-early-onset-adult-myelodysplastic-syndrome
#9
Cassandra M Hirsch, Bartlomiej P Przychodzen, Tomas Radivoyevitch, Bhumika Patel, Swapna Thota, Michael J Clemente, Yasunobu Nagata, Thomas LaFramboise, Hetty Carraway, Aziz Nazha, Mikkael A Sekeres, Hideki Makishima, Jaroslaw P Maciejewski
Myelodysplastic syndromes are typically diseases of older adults. Early onset patients may have distinct molecular and clinical features or reflect a demographic continuum. The identification of differences between early onset patients and those diagnosed at a traditional age has the potential to advance understanding of the pathogenesis of myelodysplasia and may lead to formation of distinct morphologic subcategories. We studied a cohort of 634 patients with various subcategories of myelodysplastic syndrome and secondary acute myeloid leukemia, stratifying based on age at presentation and clinical parameters...
March 2, 2017: Haematologica
https://www.readbyqxmd.com/read/28255021/no-correlation-of-intensity-of-phlebotomy-regimen-with-risk-of-thrombosis-in-polycythemia-vera-evidence-from-eclap-and-cyto-pv-clinical-trials
#10
Tiziano Barbui, Alessandra Carobbio, Arianna Ghirardi, Arianna Masciulli, Alessandro Rambaldi, Alessandro M Vannucchi
No abstract text is available yet for this article.
March 2, 2017: Haematologica
https://www.readbyqxmd.com/read/28255020/impact-of-abo-incompatibility-on-patient-outcome-in-haploidentical-hematopoietic-stem-cell-transplantation-for-acute-myeloid-leukemia-a-report-from-the-acute-leukemia-working-party-of-the-ebmt
#11
Jonathan Canaani, Bipin N Savani, Myriam Labopin, Xiao-Jun Huang, Fabio Ciceri, William Arcese, Johanna Tischer, Yener Koc, Benedetto Bruno, Zafer Gülbas, Didier Blaise, Johan Maertens, Gerhard Ehninger, Mohamad Mohty, Arnon Nagler
A significant segment of hematopoietic stem cell transplantations are performed with ABO-mismatched donors. The impact of ABO-mismatch on outcome following transplantation remains controversial and there is no published data regarding the impact of ABO-mismatch in acute myeloid leukemia patients receiving haploidentical transplantation. Using the EBMT acute leukemia working group registry we identified 837 patients who underwent haploidentical transplantation. Comparative analysis was performed between patients receiving ABO-matched versus ABO-mismatched for common clinical outcome variables...
March 2, 2017: Haematologica
https://www.readbyqxmd.com/read/28255019/a-risk-factor-analysis-of-outcomes-after-unrelated-cord-blood-transplantation-for-children-with-wiskott-aldrich-syndrome
#12
Zhanna Shekhovtsova, Carmem Bonfim, Annalisa Ruggeri, Samantha Nichele, Kristin Page, Amal AlSeraihy, Francisco Barriga, José Sánchez de Toledo Codina, Paul Veys, Jaap Jan Boelens, Karin Mellgren, Henrique Bittencourt, Tracey O' Brien, Peter J Shaw, Alicja Chybicka, Fernanda Volt, Federica Giannotti, Eliane Gluckman, Joanne Kurtzberg, Andrew R Gennery, Vanderson Rocha
Wiskott-Aldrich syndrome is a severe X-linked recessive immune deficiency disorder. A scoring system of Wiskott-Aldrich syndrome severity (0.5-5) distinguishes 2 phenotypes: X-linked thrombocytopenia and classic Wiskott-Aldrich syndrome. Hematopoietic cell transplantation is curative for Wiskott-Aldrich syndrome, however the use of unrelated umbilical cord blood transplantation has seldom been described. We analyzed umbilical cord blood transplantation outcomes for 90 patients. Median age at umbilical cord blood transplantation was 1...
March 2, 2017: Haematologica
https://www.readbyqxmd.com/read/28255018/acute-lymphoblastic-leukemia-with-aleukemic-prodrome-preleukemic-dynamics-and-possible-mechanisms-of-immunosurveillance
#13
Olga Zimmermannova, Marketa Zaliova, Anthony V Moorman, Halima Al-Shehhi, Eva Fronkova, Zuzana Zemanova, Tomas Kalina, Ajay Vora, Jan Stary, Jan Trka, Ondrej Hrusak, Jan Zuna
No abstract text is available yet for this article.
March 2, 2017: Haematologica
https://www.readbyqxmd.com/read/28255017/cdk6-contributes-to-cytoskeletal-stability-in-erythroid-cells
#14
Iris Z Uras, Ruth M Scheicher, Karoline Kollmann, Martin Glösmann, Michaela Prchal-Murphy, Anca S Tigan, Daniela A Fux, Sandro Altamura, Joana Neves, Martina Muckenthaler, Keiryn L Bennett, Stefan Kubicek, Philip W Hinds, Marieke von Lindern, Veronika Sexl
Mice lacking Cdk6 kinase activity suffer from mild anemia accompanied by elevated numbers of Ter119+ cells in the bone marrow. The animals show hardly any alterations in erythroid development, indicating that Cdk6 is not required for proliferation and maturation of erythroid cells. There is also no difference in stress erythropoiesis following hemolysis in vivo. However, Cdk6-/- erythrocytes have a shortened lifespan and are more sensitive to mechanical stress in vitro, suggesting differences in the cytoskeletal architecture...
March 2, 2017: Haematologica
https://www.readbyqxmd.com/read/28255016/prognostic-impact-of-circulating-plasma-cells-in-patients-with-multiple-myeloma-implications-for-plasma-cell-leukaemia-definition
#15
Miquel Granell, Xavier Calvo, Antoni Garcia-Guiñón, Lourdes Escoda, Eugènia Abella, Clara M Martínez, Montserrat Teixidó, Maria Teresa Gimenez, Alicia Senín, Patricia Sanz, Desirée Campoy, Ana Vicent, Leonor Arenillas, Laura Rosiñol, Jorge Sierra, Joan Blade, Carlos Fernández de Larrea
The presence of circulating plasma cells in patients with multiple myeloma is considered a marker for highly proliferative disease. In the present study, the impact of circulating plasma cells assessed by cytology on survival of patients with multiple myeloma was analysed. Wright-Giemsa stained peripheral blood smears of 482 patients with newly diagnosed myeloma or plasma cell leukaemia were reviewed and patients were classified in four categories according to the percentage of circulating plasma cells: 0%, 1-4%, 5-20% and plasma cell leukemia with the following frequencies: 382 (79...
March 2, 2017: Haematologica
https://www.readbyqxmd.com/read/28255015/distinct-molecular-genetics-of-chronic-lymphocytic-leukemia-in-taiwan-clinical-and-pathogenetic-implications
#16
Shang-Ju Wu, Chien-Ting Lin, Andreas Agathangelidis, Liang-In Lin, Yuan-Yeh Kuo, Hwei-Fang Tien, Paolo Ghia
Differences in chronic lymphocytic leukemia between Asian and the Western population is widely known. To further clarify these ethnic differences, we profiled the molecular genetics in a cohort of 83 newly diagnosed patients from Taiwan. In detail, we assessed: (i) the usage and the mutational status of the clonotypic immunoglobulin heavy-chain variable region (IgHV) genes, (ii) the presence of VH CDR3 stereotypes and (iii) TP53, NOTCH1, SF3B1, BIRC3, and MYD88 mutations. The IgHV gene repertoire was biased and distinct from that observed in the West with the most common IgHV genes being IgHV3-23, IgHV3-7, and IgHV3-48...
March 2, 2017: Haematologica
https://www.readbyqxmd.com/read/28255014/macrothrombocytopenia-and-dense-granule-deficiency-associated-with-fli1-variants-ultrastructural-and-pathogenic-features
#17
Paul Saultier, Léa Vidal, Matthias Canault, Denis Bernot, Céline Falaise, Catherine Pouymayou, Jean-Claude Bordet, Noémie Saut, Agathe Rostan, Véronique Baccini, Franck Peiretti, Marie Favier, Pauline Lucca, Jean-François Deleuze, Robert Olaso, Anne Boland, Pierre Emmanuel Morange, Christian Gachet, Fabrice Malergue, Sixtine Fauré, Anita Eckly, David-Alexandre Trégouët, Marjorie Poggi, Marie-Christine Alessi
Congenital macrothrombocytopenia is a family of rare diseases, of which a significant fraction remains to be genetically characterized. To analyze cases of unexplained thrombocytopenia, 27 individuals from a patient cohort of the Bleeding and Thrombosis Exploration Center of the University Hospital of Marseille were recruited for a high-throughput gene sequencing study. This strategy led to the identification of two novel FLI1 variants (c.1010G>A and c.1033A>G) responsible for macrothrombocytopenia. The FLI1 variant carriers platelets exhibited a defect in aggregation induced by low dose ADP, collagen and TRAP, a defect in ATP secretion, a reduced mepacrine uptake and release and a reduced CD63 expression upon TRAP stimulation...
March 2, 2017: Haematologica
https://www.readbyqxmd.com/read/28255013/histone-deacetylase-6-regulates-cytokinesis-and-erythrocyte-enucleation-through-deacetylation-of-formin-protein-mdia2
#18
Xuehui Li, Yang Mei, Bowen Yan, Eric Vitriol, Suming Huang, Peng Ji, Yi Qiu
The formin mDia2 plays a critical role in a number of cellular processes through its ability to promote nucleation and elongation of actin filaments. In erythroblasts, this includes control of cytokinesis and enucleation by regulating contractile actin ring formation. Here, we report a novel mechanism of how mDia2 is regulated: through acetylation and deacetylation at lysine 970 in formin homology 2 domain. Ectopic expression of an acetyl-mimic mDia2 mutant in mouse erythroblasts is sufficient to abolish contractile actin ring formation at the cleavage furrow and subsequent erythrocyte cytokinesis and enucleation...
March 2, 2017: Haematologica
https://www.readbyqxmd.com/read/28232372/late-effects-of-blood-and-marrow-transplantation
#19
Yoshihiro Inamoto, Stephanie J Lee
Hematopoietic cell transplantation is a curative treatment for a variety of hematologic diseases. Advances in transplantation technology have reduced early transplant-related mortality and expanded application of transplantation to older patients and to a wider variety of diseases. Management of late effects after transplantation is increasingly important for a growing number of long-term survivors that is estimated to be half a million worldwide. Many studies have shown that transplant survivors suffer from significant late effects that adversely affect morbidity, mortality, working status and quality of life...
February 23, 2017: Haematologica
https://www.readbyqxmd.com/read/28232371/c-myc-dysregulation-is-a-co-transforming-event-for-nf-%C3%AE%C2%BAb-activated-b-cells
#20
Amandine David, Nicolas Arnaud, Magali Fradet, Hélène Lascaux, Catherine Ouk-Martin, Nathalie Gachard, Ursula Zimber-Strobl, Jean Feuillard, Nathalie Faumont
While c-Myc dysregulation is constantly associated with highly proliferating B-cell tumors, NF-κB addiction is found in indolent lymphomas as well as diffuse large B-cell lymphomas either with an activated B-cell like phenotype or associated with Epstein-Barr virus. We raised the question of the effect of c-Myc in B-cells with NF-κB activated by three different inducers: Epstein-Barr Virus-latency III program, TLR-9 and CD40. Induction of c-Myc overexpression increased proliferation of Epstein-Barr Virus-latency III immortalized B-cells, an effect that was dependent on NF-κB...
February 23, 2017: Haematologica
journal
journal
24858
1
2
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"