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Avelumab for the treatment of relapsed or refractory extranodal NK/T-cell lymphoma: an open-label phase 2 study.

Seok Jin Kim, Jing Quan Lim, Yurike Laurensia, Junhun Cho, Sang Eun Yoon, Ji Young Lee, Kyung Ju Ryu, Young Hyeh Ko, Youngil Koh, Duck Cho, Soon Thye Lim, Marie Beck Enemark, Francesco D'amore, Mette Bjerre, Choon Kiat Ong, Won Seog Kim,

This study aimed to assess the efficacy and safety of treatment with avelumab, an anti-programmed death ligand 1 (PD-L1) antibody, in patients with relapsed or refractory extranodal natural killer (NK)/T-cell lymphoma (ENKTL). In this phase II trial, 21 patients with relapsed or refractory ENKTL were treated with 10 mg/kg of ... Read more >>

Blood (Blood)
[2020, :]

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Emicizumab for the treatment of acquired hemophilia A.

Paul Knoebl, Johannes Thaler, Petra Jilma, Peter Quehenberger, Karoline Veronika Gleixner, Wolfgang R Sperr,

Acquired hemophilia A (AHA) is a severe bleeding disorder caused by inhibiting autoantibodies to coagulation factor VIII (FVIII). For hemostatic treatment, bypassing agents, human or porcine FVIII are currently standard of care. Emicizumab is a bispecific, FVIII-mimetic therapeutic antibody, that reduced the annualized bleeding rates in congenital hemophiliacs. Here we ... Read more >>

Blood (Blood)
[2020, :]

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Adult blood stem cell localization reflects the abundance of reported bone marrow niche cell types and their combinations.

Konstantinos Kokkaliaris, Leo Kunz, Nina Cabezas-Wallscheid, Constantina Christodoulou, Simon Renders, Fernando Camargo, Andreas Trumpp, David T Scadden, Timm Schroeder,

The exact localization of hematopoietic stem cells (HSCs) in their native bone marrow (BM) microenvironment remains controversial, since multiple cell types have been reported to physically associate with HSCs. Here, we comprehensively quantified HSC localization with up to 4 simultaneous (9 total) BM components, in 152 full-bone sections from different ... Read more >>

Blood (Blood)
[2020, :]

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FcγRIIA expression aggravates nephritis and increases platelet activation in systemic lupus erythematosus in mice.

Imene Melki, Isabelle Allaeys, Nicolas Tessandier, Benoit Mailhot, Nathalie Cloutier, Robert A Campbell, Jesse W Rowley, David Salem, Anne Zufferey, Audrée Laroche, Tania Lévesque, Natalie Patey, Joyce Rauch, Christian Lood, Arnaud Droit, Steven E McKenzie, Kellie R Machlus, Matthew T Rondina, Steve Lacroix, Paul Fortin, Eric Boilard,

Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease characterized by deposits of immune complexes (IC) in organs and tissues. The expression of FcγRIIA by human platelets, which is their unique receptor for IgG antibodies, positions them to ideally respond to circulating IC. Whereas chronic platelet activation and thrombosis are ... Read more >>

Blood (Blood)
[2020, :]

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Post-discharge thrombosis and hemorrhage in patients with COVID-19.

Rushad Patell, Thomas Bogue, Anita G Koshy, Poorva Bindal, Mwanasha Merrill, William C Aird, Kenneth A Bauer, Jeffrey I Zwicker,

Coronavirus Disease-2019 (COVID-19) is associated with a prothrombotic state with a high incidence of thrombotic events during hospitalization; however there are limited data examining rates of thrombosis after discharge. We conducted a retrospective observational cohort study of discharged patients with confirmed COVID-19 not receiving anticoagulation. The cohort included 163 patients ... Read more >>

Blood (Blood)
[2020, :]

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Smarca5 mediated epigenetic programming facilitates fetal HSPC development in vertebrates.

Yanyan Ding, Wen Wang, Dongyuan Ma, Guixian Liang, Zhixin Kang, Yuanyuan Xue, Yifan Zhang, Lu Wang, Jian Heng, Yong Zhang, Feng Liu,

Nascent HSPCs acquire definitive hematopoietic characteristics only when they develop into fetal HSPCs; however, the mechanisms underlying fetal HSPC development are poorly understood. Here, we profiled the chromatin accessibility and transcriptional features of zebrafish nascent- and fetal HSPCs using ATAC-seq and RNA-seq and revealed dynamic changes during HSPC transition. Functional ... Read more >>

Blood (Blood)
[2020, :]

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Prostaglandin E2 stimulates cAMP signaling and re-sensitizes human leukemia cells to glucocorticoid-induced cell death.

Justine Elizabeth Roderick, Kayleigh Mary Gallagher, Leonard C Murphy, Kevin W O'Connor, Katherine Tang, Boyao Zhang, Michael Brehm, Dale L Greiner, Jun Yu, Lihua Julie Zhu, Michael R Green, Michelle A Kelliher,

Glucocorticoid (GC) resistance remains a clinical challenge in pediatric acute lymphoblastic leukemia (ALL) where response to GC is a reliable prognostic indicator. To identify GC resistance pathways, we conducted a genome-wide, survival-based, shRNA screen in murine T cell acute lymphoblastic leukemia (T-ALL) cells. Genes identified in the screen interfere with ... Read more >>

Blood (Blood)
[2020, :]

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Refractory acute graft-versus-host disease: a new working definition beyond corticosteroid refractoriness.

Mohamad Mohty, Ernst Holler, Madan Jagasia, Robert R Jenq, Florent Malard, Paul J Martin, Gerard Socié, Robert Zeiser,

Graft-versus-host disease (GvHD) remains a major limitation of allogeneic hematopoietic stem cell transplantation. Only half of patients with severe acute GvHD respond to first-line treatment with corticosteroids and, for several decades, there was no optimal second-line treatment for patients with corticosteroid-refractory acute GvHD. Ruxolitinib was recently approved for the treatment ... Read more >>

Blood (Blood)
[2020, :]

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Hepatosplenic T-cell lymphoma: A rare but challenging entity.

Barbara Pro, Pamela Blair Allen, Amir Behdad,

Hepatosplenic T-cell lymphoma (HSTCL) is a rare T-cell neoplasm that most commonly arises from a small subset of γ/δ T-cell receptor-expressing lymphocytes. HSTCL is more common in adolescent and young adults and has a rapidly progressive clinical course and poor outcome due to its refractoriness to conventional chemotherapy regimens. Approximately ... Read more >>

Blood (Blood)
[2020, :]

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Conformation-Dependent Blockage of Activated VWF Improved Outcomes of Traumatic Brain Injury in Mice.

Xin Xu, Chenyu Wang, Yingang Wu, Katie L Houck, Tristan Hilton, Ashley Zhou, Xiaoping Wu, Cha Han, Mengchen Yang, Wei Yang, Fu-Dong Shi, Moritz Stolla, Miguel A Cruz, Min Li, Jianning Zhang, Jing-Fei Dong,

Traumatic brain injury-induced coagulopathy (TBI-IC) causes life-threatening secondary intracranial bleeding. Its pathogenesis differs mechanistically from that of coagulopathy arising from extracranial injuries and hemorrhagic shock, but it remains poorly understood. We report results of a study designed to test the hypothesis that von Willebrand factor (VWF) released during acute TBI ... Read more >>

Blood (Blood)
[2020, :]

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Mechanistic insights and potential therapeutic targets for NUP98-rearranged hematologic malignancies.

Nicole L Michmerhuizen, Jeffery M Klco, Charles G Mullighan,

Nucleoporin 98 (NUP98) fusion oncoproteins are observed in a spectrum of hematologic malignancies, particularly pediatric leukemias with poor patient outcomes. While wildtype full length NUP98 is a member of the nuclear pore complex, the chromosomal translocations leading to NUP98 gene fusions involve the intrinsically disordered and N-terminal region of NUP98 ... Read more >>

Blood (Blood)
[2020, :]

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Multiple Cereblon genetic changes associate with acquired resistance to Lenalidomide or Pomalidomide in Multiple Myeloma.

Sarah Gooding, Naser Ansari-Pour, Fadi Towfic, Maria Ortiz Estevez, Philip P Chamberlain, Kao-Tai Tsai, Erin Flynt, Marissa Hirst, Dan Rozelle, Paula Dhiman, Paola Neri, Karthik Ramasamy, Nizar J Bahlis, Paresh Vyas, Anjan Thakurta,

Emergence of drug resistance to all available therapies is the major challenge to improving survival in myeloma. Cereblon (CRBN) is the essential binding protein of the widely-used IMiD and novel CelMOD drugs in myeloma, as well as certain PROTACs in development for a range of diseases. Using whole genome sequencing ... Read more >>

Blood (Blood)
[2020, :]

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Bone marrow-derived myeloid progenitors as driver mutation carriers in high- and low-risk Langerhans cell histiocytosis.

Yanling Xiao, Astrid Gs van Halteren, Xin Lei, Jelske Borst, Eline Christine Steenwijk, Tom de Wit, Joanna Grabowska, Rhianne Voogd, Paul Geraeds Kemps, Jennifer Picarsic, Cor van den Bos, Jannie Borst,

Langerhans cell histiocytosis (LCH) is a myeloid neoplasia, driven by sporadic activating mutations in the MAPK pathway. The "misguided myeloid dendritic cell (DC)" model proposes that high-risk, multisystem, risk-organ positive (MS-RO+) LCH results from driver mutation in a bone marrow (BM)-resident multipotent hematopoietic progenitor, while low-risk, MS-RO- and single system ... Read more >>

Blood (Blood)
[2020, :]

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IDENTIFICATION OF OCCULT CEREBRAL MICROBLEEDS IN ADULTS WITH IMMUNE THROMBOCYTOPENIA.

Nichola Cooper, Melanie A Morrison, Camelia Vladescu, Alice Cj Hart, Deena Paul, Amna Malik, Thomas Young, Asad Luqmani, Maria Atta, David J Sharp, James B Bussel, Adam D Waldman,

Management of symptoms and prevention of life-threatening haemorrhage in Immune thrombocytopenia (ITP) must be balanced against adverse effects of available therapies. Since current treatment guidelines based on platelet count are confounded by variable bleeding phenotypes, there is a need to identify new objective markers of disease severity for improved treatment ... Read more >>

Blood (Blood)
[2020, :]

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Post-discharge venous thromboembolism following hospital admission with COVID-19.

Lara N Roberts, Martin Brunel Whyte, Loizos Georgiou, Gerard Giron, Julia Czuprynska, Catherine Rea, Bipin Vadher, Raj Patel, Emma Gee, Roopen Arya,

The association of severe COVID-19 with an increased risk of VTE has resulted in specific guidelines for its prevention and management. The VTE risk appears highest in those with critical care admission. The need for post discharge thromboprophylaxis remains controversial and this is reflected in the conflicting recommendations of expert ... Read more >>

Blood (Blood)
[2020, :]

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Clonal Hematopoiesis: Mechanisms Driving Dominance of Stem Cell Clones.

Grant Challen, Margaret A Goodell,

The discovery of clonal hematopoiesis (CH) in older individuals has changed the way hematologists and stem cell biologists view aging. Somatic mutations accumulate in stem cells over time. While most mutations have no impact, some result in subtle functional differences that ultimately manifest in distinct stem cell behaviors. With a ... Read more >>

Blood (Blood)
[2020, :]

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Early pattern of large B-cell lymphoma with IRF4 rearrangement.

Massimo Granai, Stefano Lazzi,

Blood (Blood)
[2020, 136(6):769]

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COVID-19 and the Coombs test.

Jeanne E Hendrickson, Christopher A Tormey,

Blood (Blood)
[2020, 136(6):655-656]

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HOIP for targeting diffuse large B-cell lymphoma.

Joel L Pomerantz,

Blood (Blood)
[2020, 136(6):646-647]

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The endoplasmic reticulum protein SEC22B interacts with NBEAL2 and is required for megakaryocyte α-granule biogenesis.

Richard W Lo, Ling Li, Fred G Pluthero, Richard Leung, Koji Eto, Walter H A Kahr,

Studies of inherited platelet disorders have provided many insights into platelet development and function. Loss of function of neurobeachin-like 2 (NBEAL2) causes gray platelet syndrome (GPS), where the absence of platelet α-granules indicates NBEAL2 is required for their production by precursor megakaryocytes. The endoplasmic reticulum is a dynamic network that ... Read more >>

Blood (Blood)
[2020, 136(6):715-725]

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Human erythroleukemia genetics and transcriptomes identify master transcription factors as functional disease drivers.

Alexandre Fagnan, Frederik Otzen Bagger, Maria-Riera Piqué-Borràs, Cathy Ignacimouttou, Alexis Caulier, Cécile K Lopez, Elie Robert, Benjamin Uzan, Véronique Gelsi-Boyer, Zakia Aid, Cécile Thirant, Ute Moll, Samantha Tauchmann, Amina Kurtovic-Kozaric, Jaroslaw Maciejewski, Christine Dierks, Orietta Spinelli, Silvia Salmoiraghi, Thomas Pabst, Kazuya Shimoda, Virginie Deleuze, Hélène Lapillonne, Connor Sweeney, Véronique De Mas, Betty Leite, Zahra Kadri, Sébastien Malinge, Stéphane de Botton, Jean-Baptiste Micol, Benjamin Kile, Catherine L Carmichael, Ilaria Iacobucci, Charles G Mullighan, Martin Carroll, Peter Valent, Olivier A Bernard, Eric Delabesse, Paresh Vyas, Daniel Birnbaum, Eduardo Anguita, Loïc Garçon, Eric Soler, Juerg Schwaller, Thomas Mercher,

Acute erythroleukemia (AEL or acute myeloid leukemia [AML]-M6) is a rare but aggressive hematologic malignancy. Previous studies showed that AEL leukemic cells often carry complex karyotypes and mutations in known AML-associated oncogenes. To better define the underlying molecular mechanisms driving the erythroid phenotype, we studied a series of 33 AEL ... Read more >>

Blood (Blood)
[2020, 136(6):698-714]

Cited: 1 time

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Comprehensive analyses of B cell compartments across the human body reveal novel subsets and a gut resident memory phenotype.

Nadine Marianne Weisel, Florian J Weisel, Donna L Farber, Lisa Borghesi, Yufeng Shen, Wenji Ma, Eline T Luning Prak, Mark Shlomchik,

While human B cells have been extensively studied, most reports have used peripheral blood as a source. Here we have used a unique tissue resource derived from healthy organ donors to deeply characterize human B cell compartments across multiple tissues and donors. These data sets revealed that B cells in ... Read more >>

Blood (Blood)
[2020, :]

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Dosing-related Saturation of Toxicity and Accelerated Drug Clearance with Pegaspargase Treatment.

Yiwei Liu, John Carl Panetta, Wenjian Yang, Seth E Karol, Cheng Cheng, Jun J Yang, William E Evans, Hiroto Inaba, Ching-Hon Pui, Sima Jeha, Mary V Relling,

Blood (Blood)
[2020, :]

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Pyruvate kinase deficiency in a newborn with extramedullary hematopoiesis in the skin.

Richard A Voit, Rachael F Grace,

Blood (Blood)
[2020, 136(6):770]

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Erythroleukemia: all roads lead to GATA1?

John Strouboulis,

Blood (Blood)
[2020, 136(6):648-649]

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