From www.bloodjournal.org by guest on February 6, 2015. For personal use only. 29 JANUARY 2015 x VOLUME 125, NUMBER 5 CONTENTS COVER FIGURE Cytoskeleton disorganization caused by the R46W mutation of a-actinin 1. ACTN1 is a gene recently identiﬁed as responsible for a new, autosomal-dominant form of inherited thrombocytopenia, ACTN1-related thrombocytopenia. Expression of the mutant R46W form of a-actinin 1 (green) in human ﬁbroblasts causes complete disorganization of the cytoskeleton with actin (red) no longer organized in ﬁlaments, which are instead observed in surrounding untransfected cells. See the article by Bottega et al on page 869. INSIDE BLOOD COMMENTARIES 741 There’s no such fool like an immunosenescent fool Markus G. Seidel Comment on Stepensky et al, page 753 742 Dissecting complement blockade for clinic use Antonio M. Risitano Comment on Peffault de Latour et al, page 775 744 The off-target effects of nonspeciﬁc NK cells Dean A. Lee Comment on Shah et al, page 784 745 Blind men and an elephant Shaji Kumar Comment on Pawlyn et al, page 831 747 Proplatelets slip slidin’ away Alexandra Mazharian and Yotis A. Senis Comment on Bender et al, page 860 748 Inherited thrombocytopenias: the beat goes on A. Koneti Rao and Natthapol Songdej Comment on Bottega et al, page 869 750 PI3Kb inhibition: all that glitters is not gold Mauro Torti Comment on Laurent et al, page 881 PLENARY PAPER 753 Early-onset Evans syndrome, immunodeﬁciency, and premature immunosenescence associated with tripeptidyl-peptidase II deﬁciency Polina Stepensky, Anne Rensing-Ehl, Ruth Gather, Shoshana Revel-Vilk, Ute Fischer, Schaﬁq Nabhani, Fabian Beier, Tim H. Br¨ummendorf, Sebastian Fuchs, Simon Zenke, Elke Firat, Vered Molho Pessach, Arndt Borkhardt, Mirzokhid Rakhmanov, B¨arbel Keller, Klaus Warnatz, Hermann Eibel, Gabriele Niedermann, Orly Elpeleg, and Stephan Ehl BLOOD SPOTLIGHT 762 Fcg-receptor–mediated trogocytosis impacts mAb-based therapies: historical precedence and recent developments Ronald P. Taylor and Margaret A. Lindorfer v BLOOD, 29 JANUARY 2015 x VOLUME 125, NUMBER 5 CONTINUED ON vi From www.bloodjournal.org by guest on February 6, 2015. For personal use only. HOW I TREAT 767 How I treat the older patient with acute myeloid leukemia Gert Ossenkoppele and Bob L¨owenberg CLINICAL TRIALS AND OBSERVATIONS 775 Assessing complement blockade in patients with paroxysmal nocturnal hemoglobinuria receiving eculizumab R´egis Peffault de Latour, V´eronique Fremeaux-Bacchi, Rapha¨el Porcher, Ali´enor Xhaard, J´er´emie Rosain, Diana Cadena Castaneda, Paula Vieira-Martins, St´ephane Roncelin, Paula Rodriguez-Otero, Aur´elie Plessier, Flore Sicre de Fontbrune, Sarah Abbes, Marie Robin, and G´erard Soci´e 784 Acute GVHD in patients receiving IL-15/4-1BBL activated NK cells following T-cell–depleted stem cell transplantation Nirali N. Shah, Kristin Baird, Cynthia P. Delbrook, Thomas A. Fleisher, Mark E. Kohler, Shakuntala Rampertaap, Kimberly Lemberg, Carolyn K. Hurley, David E. Kleiner, Melinda S. Merchant, Stefania Pittaluga, Marianna Sabatino, David F. Stroncek, Alan S. Wayne, Hua Zhang, Terry J. Fry, and Crystal L. Mackall HEMATOPOIESIS AND STEM CELLS 793 Bone marrow skeletal stem/progenitor cell defects in dyskeratosis congenita and telomere biology disorders Arun Balakumaran, Prasun J. Mishra, Edyta Pawelczyk, Sayuri Yoshizawa, Brian J. Sworder, Natasha Cherman, Sergei A. Kuznetsov, Paolo Bianco, Neelam Giri, Sharon A. Savage, Glenn Merlino, Bogdan Dumitriu, Cynthia E. Dunbar, Neal S. Young, Blanche P. Alter, and Pamela G. Robey IMMUNOBIOLOGY 803 A crucial role for the homeodomain transcription factor Hhex in lymphopoiesis Jacob T. Jackson, Chayanica Nasa, Wei Shi, Nicholas D. Huntington, Clifford W. Bogue, Warren S. Alexander, and Matthew P. McCormack 815 Brief Report Histone deacetylase inhibition regulates inﬂammation and enhances Tregs after allogeneic hematopoietic cell transplantation in humans Sung Won Choi, Erin Gatza, Guoqing Hou, Yaping Sun, Joel Whitﬁeld, Yeohan Song, Katherine Oravecz-Wilson, Isao Tawara, Charles A. Dinarello, and Pavan Reddy LYMPHOID NEOPLASIA 820 Mer tyrosine kinase promotes the survival of t(1;19)-positive acute lymphoblastic leukemia (ALL) in the central nervous system (CNS) Sarah Krause, Christian Pfeiffer, Susanne Strube, Ameera Alsadeq, Henning Fedders, Christian Vokuhl, Sonja Loges, Jonas Waizenegger, Isabel Ben-Batalla, Gunnar Cario, Anja M¨oricke, Martin Stanulla, Martin Schrappe, and Denis M. Schewe 831 Coexistent hyperdiploidy does not abrogate poor prognosis in myeloma with adverse cytogenetics and may precede IGH translocations Charlotte Pawlyn, Lorenzo Melchor, Alex Murison, Christopher P. Wardell, Annamaria Brioli, Eileen M. Boyle, Martin F. Kaiser, Brian A. Walker, Dil B. Begum, Nasrin B. Dahir, Paula Proszek, Walter M. Gregory, Mark T. Drayson, Graham H. Jackson, Fiona M. Ross, Faith E. Davies, and Gareth J. Morgan 841 Integrin avb3 acting as membrane receptor for thyroid hormones mediates angiogenesis in malignant T cells Florencia Cayrol, Mar´ıa Celeste D´ıaz Flaqu´e, Tharu Fernando, Shao Ning Yang, Helena Andrea Sterle, Marcela Bolontrade, Mariana Amor´os, Blanca Isse, Ricardo Norberto Far´ıas, Haelee Ahn, Ye F. Tian, Fabrizio Tabb`o, Ankur Singh, Giorgio Inghirami, Leandro Cerchietti, and Graciela Alicia Cremaschi vi BLOOD, 29 JANUARY 2015 x VOLUME 125, NUMBER 5 CONTINUED ON viii From www.bloodjournal.org by guest on February 6, 2015. For personal use only. 852 Brief Report Suppression of T-cell lymphomagenesis in mice requires PTEN phosphatase activity Ryan H. Newton, Yu Lu, Antonella Papa, Greg H. Whitcher, Youn-Jung Kang, Catherine Yan, Pier Paolo Pandolﬁ, and Laurence A. Turka 856 Brief Report Not all IGHV3-21 chronic lymphocytic leukemias are equal: prognostic considerations Panagiotis Baliakas, Andreas Agathangelidis, Anastasia Hadzidimitriou, Lesley-Ann Sutton, Eva Minga, Athina Tsanousa, Lydia Scarf`o, Zadie Davis, Xiao-Jie Yan, Tait Shanafelt, Karla Plevova, Yorick Sandberg, Fie Juhl Vojdeman, Myriam Boudjogra, Tatiana Tzenou, Maria Chatzouli, Charles C. Chu, Silvio Veronese, Anne Gardiner, Larry Mansouri, Karin E. Smedby, Lone Bredo Pedersen, Denis Moreno, Kirsten Van Lom, V´eronique Giudicelli, Hana Skuhrova Francova, Florence Nguyen-Khac, Panagiotis Panagiotidis, Gunnar Juliusson, Lefteris Angelis, Achilles Anagnostopoulos, Marie-Paule Lefranc, Monica Facco, Livio Trentin, Mark Catherwood, Marco Montillo, Christian H. Geisler, Anton W. Langerak, Sarka Pospisilova, Nicholas Chiorazzi, David Oscier, Diane F. Jelinek, Nikos Darzentas, Chrysoula Belessi, Frederic Davi, Paolo Ghia, Richard Rosenquist, and Kostas Stamatopoulos PLATELETS AND THROMBOPOIESIS 860 Microtubule sliding drives proplatelet elongation and is dependent on cytoplasmic dynein Markus Bender, Jonathan N. Thon, Allen J. Ehrlicher, Stephen Wu, Linas Mazutis, Emoke Deschmann, Martha Sola-Visner, Joseph E. Italiano, and John H. Hartwig 869 Brief Report ACTN1-related thrombocytopenia: identiﬁcation of novel families for phenotypic characterization Roberta Bottega, Caterina Marconi, Michela Faleschini, Gabriele Baj, Claudia Cagioni, Alessandro Pecci, Tommaso Pippucci, Ugo Ramenghi, Simonetta Pardini, Loretta Ngu, Carlo Baronci, Shinji Kunishima, Carlo L. Balduini, Marco Seri, Anna Savoia, and Patrizia Noris RED CELLS, IRON, AND ERYTHROPOIESIS 873 Hepcidin is suppressed by erythropoiesis in hemoglobin E b-thalassemia and b-thalassemia trait Emma Jones, Sant-Rayn Pasricha, Angela Allen, Patricia Evans, Chris A. Fisher, Katherine Wray, Anuja Premawardhena, Dyananda Bandara, Ashok Perera, Craig Webster, Pamela Sturges, Nancy F. Olivieri, Timothy St. Pierre, Andrew E. Armitage, John B. Porter, David J. Weatherall, and Hal Drakesmith THROMBOSIS AND HEMOSTASIS 881 Platelet PI3Kb and GSK3 regulate thrombus stability at a high shear rate Pierre-Alexandre Laurent, Sonia S´everin, B´eatrice Hechler, Bart Vanhaesebroeck, Bernard Payrastre, and Marie-Pierre Gratacap BLOOD WORK 889 “Cup-like” blasts in acute myeloid leukemia with FLT3 and NPM1 mutations Aditi Vidholia and Madhu P. Menon ERRATA viii 890 Irving J, Matheson E, Minto L, et al. Ras pathway mutations are prevalent in relapsed childhood acute lymphoblastic leukemia and confer sensitivity to MEK inhibition. Blood. 2014;124(23):3420-3430. 890 Boudreau LH, Duchez A-C, Cloutier N, et al. Platelets release mitochondria serving as substrate for bactericidal group IIA-secreted phospholipase A2 to promote inﬂammation. Blood. 2014;124(14):2173-2183. BLOOD, 29 JANUARY 2015 x VOLUME 125, NUMBER 5 CONTINUED ON ix From www.bloodjournal.org by guest on February 6, 2015. For personal use only. OTHER DEPARTMENTS iiib Classiﬁeds SUBMISSION INSTRUCTIONS All manuscripts, including ﬁgures, should be submitted electronically at http://submit.bloodjournal.org to Editor-in-Chief Bob L¨owenberg, MD, PhD. Before submitting your paper, review Blood’s Author Guide at http://bloodjournal.hematologylibrary.org/site/authors/ authorguide.xhtml. If you need help during the submission process, contact the Editorial Ofﬁce by phone at 202-776-0548 or via e-mail at [email protected]. ix BLOOD, 29 JANUARY 2015 x VOLUME 125, NUMBER 5 From www.bloodjournal.org by guest on February 6, 2015. For personal use only. 2015 125: 741-890 125 (5) Updated information and services can be found at: http://www.bloodjournal.org/content/125/5.full.html Articles on similar topics can be found in the following Blood collections Information about reproducing this article in parts or in its entirety may be found online at: http://www.bloodjournal.org/site/misc/rights.xhtml#repub_requests Information about ordering reprints may be found online at: http://www.bloodjournal.org/site/misc/rights.xhtml#reprints Information about subscriptions and ASH membership may be found online at: http://www.bloodjournal.org/site/subscriptions/index.xhtml Blood (print ISSN 0006-4971, online ISSN 1528-0020), is published weekly by the American Society of Hematology, 2021 L St, NW, Suite 900, Washington DC 20036. Copyright 2011 by The American Society of Hematology; all rights reserved.