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Jae-Ho Yoon, Sung-Soo Park, Young-Woo Jeon, Sung-Eun Lee, Byung Sik Cho, Ki-Seong Eom, Yoo-Jin Kim, Hee-Je Kim, Seok Lee, Chang-Ki Min, Seok-Goo Cho, Jong Wook Lee
Hemophagocytic lymphohistiocytosis is an overwhelming systemic inflammatory process that is life-threatening if not treated appropriately. We analyzed prognostic factors of secondary hemophagocytic lymphohistiocytosis excluding malignancy. In this retrospective study, we analyzed 126 adult cases between 2001 and 2017. Treatment was based on dexamethasone with or without etoposide and cyclosporine. Patients who achieved complete response at 4 weeks were defined as early-stable responders, those who failed to achieve complete response but showed continuous improvement until 8 weeks as late responders, those whose conditions waxed and waned until 8 weeks as unstable responders...
September 13, 2018: Haematologica
Henk Schonewille, Jon J van Rood, Esther P Verduin, Leo M G van de Watering, Geert W Haasnoot, Frans H J Claas, Dick Oepkes, Enrico Lopriore, Anneke Brand
The observation, by Ray Owen and colleagues in 1954, that D-negative women were less likely to form anti-D antibodies against their D-positive fetus if their mother possessed the D-antigen, was not found in all later studies. We hypothesized that breastfeeding, received by the mother, may affect her immunity against non-inherited maternal red blood cell antigens. We studied a cohort of 125 grandmother-mother-child combinations, from a follow-up study of mothers after intrauterine transfusion of the fetus for alloimmune hemolytic disease...
September 13, 2018: Haematologica
Andrea Biondi, Gunnar Cario, Paola De Lorenzo, Anders Castor, Valentino Conter, Veronica Leoni, Virginie Gandemer, Rob Pieters, Jan Stary, Gabriele Escherich, Myriam Campbell, Andishe Attarbaschi, Chi-Kong Li, Ajay Vora, Jutta Bradtke, Vaskar Saha, Maria Grazia Valsecchi, Martin Schrappe
No abstract text is available yet for this article.
September 13, 2018: Haematologica
Eduard J van Beers, Stephanie van Straaten, D Holmes Morton, Wilma Barcellini, Stefan W Eber, Bertil Glader, Hassan M Yaish, Satheesh Chonat, Janet L Kwiatkowski, Jennifer A Rothman, Mukta Sharma, Ellis J Neufeld, Sujit Sheth, Jenny M Despotovic, Nina Kollmar, Dagmar Pospisilova, Christine M Knoll, Kevin Kuo, Yves D Pastore, Alexis A Thompson, Peter E Newburger, Yaddanapudi Ravindranath, Winfred C Wang, Marcin W Wlodarski, Heng Wang, Susanne Holzhauer, Vicky R Breakey, Madeleine Verhovsek, Joachim Kunz, Melissa A McNaull, Melissa J Rose, Heather A Bradeen, Kathryn Addonizio, Anran Li, Hasan Al-Sayegh, Wendy B London, Rachael F Grace
No abstract text is available yet for this article.
September 13, 2018: Haematologica
Marjolijn C J Jongmans, Illja J Diets, Paola Quarello, Emanuela Garelli, Roland P Kuiper, Rolph Pfundt
No abstract text is available yet for this article.
September 13, 2018: Haematologica
Elisa Taiana, Domenica Ronchetti, Vanessa Favasuli, Katia Todoerti, Martina Manzoni, Nicola Amodio, Pierfrancesco Tassone, Luca Agnelli, Antonino Neri
No abstract text is available yet for this article.
September 13, 2018: Haematologica
Mélanie Gaignage, Reece G Marillier, Perrine M Cochez, Laure Dumoutier, Catherine Uyttenhove, Jean-Paul Coutelier, Jacques Van Snick
In spite of considerable therapeutic progress, acute graft-versus-host disease still limits allogeneic hematopoietic cell transplantation. We recently reported that mouse infection with nidovirus lactate dehydrogenase elevating virus impairs disease in non-conditioned B6D2F1 recipients of parental B6 spleen cells. As this virus activates TLR7, we tested pharmacologic TLR7 ligand R848 in this model and observed complete survival if donor and recipients were treated before transplantation. Mixed lymphocyte culture performed 48 h after R848-treatment of normal mice demonstrated that both T cell allo-responsiveness and antigen presentation by CD11b+ and CD8α+ dendritic cells were inhibited...
September 13, 2018: Haematologica
Bianca Schuhmacher, Julia Bein, Tobias Rausch, Vladimir Benes, Thomas Tousseyn, Martine Vornanen, Maurilio Ponzoni, Lorenz Thurner, Randy Gascoyne, Christian Steidl, Ralf Küppers, Martin-Leo Hansmann, Sylvia Hartmann
T-cell/histiocyte-rich large B-cell lymphoma is a rare aggressive lymphoma showing histopathological overlap with nodular lymphocyte predominant Hodgkin lymphoma. Despite differences in tumor microenvironment and clinical behavior, the tumor cells of both entities show remarkable similarities, suggesting that both lymphomas might represent a spectrum of the same disease. To address this issue, we investigated if these entities share mutations. Ultra-deep targeted resequencing of 6 typical and 11 histopathological variants of nodular lymphocyte predominant Hodgkin lymphoma, and 9 T-cell/histiocyte-rich large B-cell lymphoma cases revealed that genes recurrently mutated in nodular lymphocyte predominant Hodgkin lymphoma are affected by mutations at similar frequencies in T-cell/histiocyte-rich large B-cell lymphoma...
September 13, 2018: Haematologica
Elisabeth K M Mack, André Marquardt, Danny Langer, Petra Ross, Alfred Ultsch, Michael G Kiehl, Hildegard I D Mack, Torsten Haferlach, Andreas Neubauer, Cornelia Brendel
Differential induction therapy of all subtypes of Acute Myeloid Leukemia other than Acute Promyelocytic Leukemia is impeded by the long time required to complete complex and diverse cytogenetic and molecular genetic analyses for risk stratification or targeted treatment decisions. Here, we describe a reliable, rapid and sensitive diagnostic approach that combines karyotyping and mutational screening in a single integrated next generation sequencing assay. Numerical karyotyping was performed by low coverage whole genome sequencing followed by copy number variation analysis using a novel algorithm based on in silico-generated reference karyotypes...
September 6, 2018: Haematologica
Jyoti Naik, Maria Themeli, Regina de Jong-Korlaar, Ruud W J Ruiter, Pino J Poddighe, Huipin Yuan, Joost D de Bruijn, Gert J Ossenkoppele, Sonja Zweegman, Linda Smit, Tuna Mutis, Anton C M Martens, Niels W C J van de Donk, Richard W J Groen
September 6, 2018: Haematologica
Paolo Strati, Michelle Fanale, Yasuhiro Oki, Francesco Turturro, Luis E Fayad, Nancy L Bartlett, Douglas E Gladstone, Yvette L Kasamon, Carol S Portlock, Wyndham H Wilson, Andre Goy, Anas Younes, Hun Ju Lee
No abstract text is available yet for this article.
September 6, 2018: Haematologica
Anna Lucia Fedullo, Monica Messina, Loredana Elia, Alfonso Piciocchi, Valentina Gianfelici, Alessia Lauretti, Stefano Soddu, Maria Cristina Puzzolo, Clara Minotti, Felicetto Ferrara, Bruno Martino, Patrizia Chiusolo, Valeria Calafiore, Stefania Paolini, Marco Vignetti, Antonella Vitale, Anna Guarini, Robin Foà, Sabina Chiaretti
To shed light into the molecular basis of Ph+ acute lymphoblastic leukemia and to investigate the prognostic role of additional genomic lesions, we analyzed copy number aberrations using the Cytoscan HD Array in 116 newly diagnosed adult Ph+ acute lymphoblastic leukemia patients enrolled in four different GIMEMA protocols, all based on a chemotherapy-free induction strategy. This analysis showed that Ph+ acute lymphoblastic leukemia patients carry 7.8 lesions/case on average, with deletions outnumbering gains (88% vs 12%)...
September 6, 2018: Haematologica
Toby A Eyre, Harriet S Walter, Sunil Iyengar, George Follows, Matthew Cross, Christopher P Fox, Andrew Hodson, Josh Coats, Santosh Narat, Nick Morley, Martin J S Dyer, Graham P Collins
No abstract text is available yet for this article.
September 6, 2018: Haematologica
Jo Caers, Laurent Garderet, K Martin Kortüm, Michael E O'Dwyer, Niels W C J van de Donk, Mascha Binder, Sandra Maria Dold, Francesca Gay, Jill Corre, Yves Beguin, Heinz Ludwig, Alessandra Larocca, Christoph Driessen, Meletios A Dimopoulos, Mario Boccadoro, Martin Gramatzki, Sonja Zweegman, Hermann Einsele, Michele Cavo, Hartmut Goldschmidt, Pieter Sonneveld, Michel Delforge, Holger W Auner, Evangelos Terpos, Monika Engelhardt
The diagnosis of multiple myeloma can be challenging, even for experienced physicians, and requires close collaboration between multiple disciplines (orthopedics, radiology, nuclear medicine, radiation therapy, hematology and oncology) before the final diagnosis of myeloma is made. The definition of multiple myeloma is based on the presence of clinical, biochemical, histo-pathological, and radiological disease markers. At presentation and during follow-up, specific tests are needed for exact disease characterization and correct diagnosis...
August 31, 2018: Haematologica
Julia Stomper, Gabriele Ihorst, Stefan Suciu, Philipp N Sander, Heiko Becker, Pierre W Wijermans, Christoph Plass, Dieter Weichenhan, Emmanuel Bissé, Rainer Claus, Michael Lübbert
Hematologic responses to hypomethylating agents in myelodysplastic syndrome and acute myeloid leukemia patients are often delayed. Fetal hemoglobin is a potential novel biomarker for response: recently, we could demonstrate that its elevation prior to decitabine treatment was associated with superior outcome. We now asked whether early fetal hemoglobin induction during decitabine treatment also had prognostic value, and investigated the potential of decitabine for in vitro induction of erythroid differentiation and fetal hemoglobin expression...
August 31, 2018: Haematologica
Kazuya Ishiguro, Hiroshi Kitajima, Takeshi Niinuma, Tadao Ishida, Reo Maruyama, Hiroshi Ikeda, Toshiaki Hayashi, Hajime Sasaki, Hideki Wakasugi, Koyo Nishiyama, Tetsuya Shindo, Eiichiro Yamamoto, Masahiro Kai, Yasushi Sasaki, Takashi Tokino, Hiroshi Nakase, Hiromu Suzuki
Epigenetic alterations play an important role in the pathogenesis in multiple myeloma, but their biological and clinical relevance is not fully understood. Here, we show that DOT1L, which catalyzes methylation of histone H3 lysine 79, is required for myeloma cell survival. DOT1L expression levels were higher in monoclonal gammopathy of undetermined significance and smoldering multiple myeloma than in normal plasma cells. Treatment with a DOT1L inhibitor induced cell cycle arrest and apoptosis in myeloma cells, and strongly suppressed cell proliferation in vitro...
August 31, 2018: Haematologica
Marie Luise Hütter-Krönke, Walter Fiedler, Andrea Kündgen, Jürgen Krauter, Marie von Lilienfeld-Toal, Hartmut Döhner, Richard Schlenk
No abstract text is available yet for this article.
August 31, 2018: Haematologica
Kentaro Ohki, Nobutaka Kiyokawa, Yuya Saito, Shinsuke Hirabayashi, Kazuhiko Nakabayashi, Hitoshi Ichikawa, Yukihide Momozawa, Kohji Okamura, Ai Yoshimi, Hiroko Ogata-Kawata, Hiromi Sakamoto, Motohiro Kato, Keitaro Fukushima, Daisuke Hasegawa, Hiroko Fukushima, Masako Imai, Ryosuke Kajiwara, Takashi Koike, Isao Komori, Atsushi Matsui, Makiko Mori, Koichi Moriwaki, Yasushi Noguchi, Myoung-Ja Park, Takahiro Ueda, Shohei Yamamoto, Koichi Matsuda, Teruhiko Yoshida, Kenji Matsumoto, Kenichiro Hata, Michiaki Kubo, Yoichi Matsubara, Hiroyuki Takahashi, Takashi Fukushima, Yasuhide Hayashi, Katsuyoshi Koh, Atsushi Manabe, Akira Ohara
Fusion genes involving MEF2D have recently been identified in precursor B-cell acute lymphoblastic leukemia, mutually exclusive of the common risk stratifying genetic abnormalities, although their true incidence and associated clinical characteristics remains unknown. We identified 16 acute lymphoblastic leukemia cases and 1 lymphoma case harboring MEF2D fusions, including MEF2D-BCL9 (n=10), MEF2D-HNRNPUL1 (n=6) and one novel MEF2D-HNRNPH1 fusion. The incidence of MEF2D fusions overall was 2.4% among consecutive B-cell acute lymphoblastic leukemia patients enrolled onto a single clinical trial...
August 31, 2018: Haematologica
Laura W Dillon, Sheida Hayati, Gregory W Roloff, Ilker Tunc, Mehdi Pirooznia, Antonina Mitrofanova, Christopher S Hourigan
Great effort is spent on developing therapies to improve the dire outcomes of those diagnosed with acute myeloid leukemia, while the methods for quantifying response to therapeutic intervention have lacked sensitivity. As a result, those patients achieving a complete remission remain at risk of subsequent relapse due to disease persistence not evident by conventional cytomorphological methods. Improved risk stratification is possible based on tests designed to detect this residual leukemic burden (measurable residual disease)...
August 31, 2018: Haematologica
Chi Young Ok, Sanam Loghavi, Dawen Sui, Peng Wei, Rashmi Kanagal-Shamanna, C Cameron Yin, Zhuang Zuo, Mark J Routbort, Guilin Tang, Zhenya Tang, Jeffrey L Jorgensen, Rajyalakshmi Luthra, Farhad Ravandi, Hagop M Kantarjian, Courtney D DiNardo, L Jeffrey Medeiros, Sa A Wang, Keyur P Patel
Persistence of IDH1 or IDH2 mutations in remission bone marrow specimens of patients with acute myeloid leukemia has been observed, but the clinical impact of these mutations is not well known. In this study, we collected 80 acute myeloid leukemia patients with known IDH1 R132 or IDH2 R140/R172 mutations and assessed their bone marrow at time of remission to determine the potential impact of persistent IDH1/2 mutations. Approximately 40% of acute myeloid leukemia patients with standard treatment in this cohort had persistent mutations in IDH1/2...
August 31, 2018: Haematologica
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