Review Articles




The molecular basis for the prothrombotic state in sickle cell disease

Arun S. Shet, Maria A. Lizarralde-Iragorri, Rakhi P. Naik


Clonal hematopoietic mutations linked to platelet traits and the risk of thrombosis or bleeding

Alicia Veninga, Ilaria De Simone, Johan W.M. Heemskerk, Hugo ten Cate, Paola E.J. van der Meijden


Acquired von Willebrand syndrome: focused for hematologists

Massimo Franchini, Pier Mannuccio Mannucci


Potential targeting of FLT3 AML

Alexander J. Ambinder, Mark Levis


Multiple myeloma with central nervous system relapse

Philip A. Egan, Patrick T Elder, W. Ian Deighan, Sheila J.M. O’Connor, H. Denis Alexander


The variable manifestations of disease in pyruvate kinase deficiency and their management

Hanny Al-Samkari, Eduard J. van Beers, Kevin H.M. Kuo, Wilma Barcellini, Paola Bianchi, Andreas Glenthøj, María del Mar Mañú Pereira, Richard van Wijk, Bertil Glader, Rachael F. Grace



Geriatric assessment in older patients with a hematologic malignancy: a systematic review

Ellen R.M. Scheepers, Ariel M. Vondeling, Noortje Thielen, René van der Griend, Reinhard Stauder, Marije E. Hamaker




Bone marrow niche dysregulation in myeloproliferative neoplasms

Natalia Curto-Garcia, Claire Harrison, Donal P McLornan



How I curate: applying American Society of Hematology-Clinical Genome Resource Myeloid Malignancy Variant Curation Expert Panel rules for RUNX1 variant curation for germline predisposition to myeloid malignancies

David Wu, Xi Luo, Simone Feurstein, Chimene Kesserwan, Shruthi Mohan, Daniel E. Pineda-Alvarez, Lucy A. Godley, on behalf of the collaborative group of the American Society of Hematology - Clinical Genome Resource Myeloid Malignancy Variant Curation Expert Panel


Glanzmann thrombasthenia: genetic basis and clinical correlates

Juliana Perez Botero, Kristy Lee, Brian R Branchford, Paul F Bray, Kathleen Freson, Michele P. Lambert, Minjie Luo, Shruthi Mohan, Justyne E. Ross, Wolfgang Bergmeier, Jorge Di Paola


An international registry of patients with plasminogen deficiency (HISTORY)

Amy D. Shapiro, Marzia Menegatti, Roberta Palla, Marco Boscarino, Christopher Roberson, Paolo Lanzi, Joel Bowen, Charles Nakar, Isaac A. Janson, Flora Peyvandi


Seronegative antiphospholipid syndrome: refining the value of “non-criteria” antibodies for diagnosis and clinical management

Pasquale Pignatelli, Evaristo Ettorre, Danilo Menichelli, Arianna Pani, Francesco Violi, Daniele Pastori


Innate immune cells, major protagonists of sickle cell disease pathophysiology

Slimane Allali, Thiago Trovati Maciel, Olivier Hermine, Mariane de Montalembert


Fibrin(ogen) in human disease: both friend and foe

Rui Vilar, Richard J. Fish, Alessandro Casini, Marguerite Neerman-Arbez


Understanding intrinsic hematopoietic stem cell aging

Eva Mejia-Ramirez, Maria Carolina Florian



MYD88 in the driver’s seat of B-cell lymphomagenesis: from molecular mechanisms to clinical implications

Ruben A.L. de Groen, Anne M.R. Schrader, Marie José Kersten, Steven T. Pals, Joost S.P. Vermaat






Updates on the hematologic tumor microenvironment and its therapeutic targeting

Dorian Forte, Daniela S. Krause, Michael Andreeff, Dominique Bonnet, Simón Méndez-Ferrer








Thrombopoietin receptor agonists: ten years later

Waleed Ghanima, Nichola Cooper, Francesco Rodeghiero, Bertrand Godeau, James B. Bussel




CRISPR to fix bad blood: a new tool in basic and clinical hematology

Elisa González-Romero, Cristina Martínez-Valiente, Cristian García-Ruiz, Rafael P. Vázquez-Manrique, José Cervera, Alejandra Sanjuan-Pla



State-of-the-art review: allogeneic stem cell transplantation for myelofibrosis in 2019

Donal P. McLornan, Ibrahim Yakoub-Agha, Marie Robin, Yves Chalandon, Claire N. Harrison, Nicolaus Kroger



IgG4-related disease: what a hematologist needs to know

Luke Y.C. Chen, Andre Mattman, Michael A. Seidman, Mollie N. Carruthers


CD30-positive primary cutaneous lymphoproliferative disorders: molecular alterations and targeted therapies

Lucia Prieto-Torres, Socorro M. Rodriguez-Pinilla, Arantza Onaindia, Mariano Ara, Luis Requena, Miguel Á. Piris





Osteogenic niche in the regulation of normal hematopoiesis and leukemogenesis

Phuong M. Le, Michael Andreeff, Venkata Lokesh Battula


TP53 aberrations in chronic lymphocytic leukemia: an overview of the clinical implications of improved diagnostics

Elias Campo, Florence Cymbalista, Paolo Ghia, Ulrich Jäger, Sarka Pospisilova, Richard Rosenquist, Anna Schuh, Stephan Stilgenbauer


Preclinical human models and emerging therapeutics for advanced systemic mastocytosis

Michel Arock, Ghaith Wedeh, Gregor Hoermann, Siham Bibi, Cem Akin, Barbara Peter, Karoline V. Gleixner, Karin Hartmann, Joseph H. Butterfield, Dean D. Metcalfe, Peter Valent



Normal and pathological erythropoiesis in adults: from gene regulation to targeted treatment concepts

Peter Valent, Guntram Büsche, Igor Theurl, Iris Z. Uras, Ulrich Germing, Reinhard Stauder, Karl Sotlar, Wolfgang Füreder, Peter Bettelheim, Michael Pfeilstöcker, Rainer Oberbauer, Wolfgang R. Sperr, Klaus Geissler, Jürg Schwaller, Richard Moriggl, Marie C. Béné, Ulrich Jäger, Hans-Peter Horny, Olivier Hermine


Cardiovascular adverse events in modern myeloma therapy – Incidence and risks. A review from the European Myeloma Network (EMN) and Italian Society of Arterial Hypertension (SIIA)

Sara Bringhen, Alberto Milan, Claudio Ferri, Ralph Wäsch, Francesca Gay, Alessandra Larocca, Marco Salvini, Evangelos Terpos, Hartmut Goldschmidt, Michele Cavo, Maria Teresa Petrucci, Heinz Ludwig, Holger W. Auner, Jo Caers, Martin Gramatzki, Mario Boccadoro, Hermann Einsele, Pieter Sonneveld, Monika Engelhardt




Dissecting the pathophysiology of immune thrombotic thrombocytopenic purpura: interplay between genes and environmental triggers

Johana Hrdinová, Silvia D’Angelo, Nuno A. G. Graça, Bogac Ercig, Karen Vanhoorelbeke, Agnès Veyradier, Jan Voorberg, Paul Coppo



The many faces of IKZF1 in B-cell precursor acute lymphoblastic leukemia

René Marke, Frank N. van Leeuwen, Blanca Scheijen


Extracellular vesicles in the hematopoietic microenvironment

John T. Butler, Sherif Abdelhamed, Peter Kurre


From transplant to novel cellular therapies in multiple myeloma: European Myeloma Network guidelines and future perspectives

Francesca Gay, Monika Engelhardt, Evangelos Terpos, Ralph Wäsch, Luisa Giaccone, Holger W. Auner, Jo Caers, Martin Gramatzki, Niels van de Donk, Stefania Oliva, Elena Zamagni, Laurent Garderet, Christian Straka, Roman Hajek, Heinz Ludwig, Herman Einsele, Meletios Dimopoulos, Mario Boccadoro, Nicolaus Kröger, Michele Cavo, Hartmut Goldschmidt, Benedetto Bruno, Pieter Sonneveld


The prothrombotic state in paroxysmal nocturnal hemoglobinuria: a multifaceted source

Barnaby Peacock-Young, Fraser L. Macrae, Darren J. Newton, Anita Hill, Robert A S Ariëns



Incidence and management of toxicity associated with ibrutinib and idelalisib: a practical approach

Iris de Weerdt, Suzanne M. Koopmans, Arnon P. Kater, Michel van Gelder



Genetic background and evolution of relapses in aggressive B-cell lymphomas

Darius Juskevicius, Stephan Dirnhofer, Alexandar Tzankov



Late effects of blood and marrow transplantation

Yoshihiro Inamoto, Stephanie J. Lee


Is immunotherapy here to stay in multiple myeloma?

Paula Rodríguez-Otero, Bruno Paiva, Monika Engelhardt, Felipe Prósper, Jesús F. San Miguel


Propagation of thrombosis by neutrophils and extracellular nucleosome networks

Susanne Pfeiler, Konstantin Stark, Steffen Massberg, Bernd Engelmann



Anti-thymocyte globulin as graft-versus-host disease prevention in the setting of allogeneic peripheral blood stem cell transplantation: a review from the Acute Leukemia Working Party of the European Society for Blood and Marrow Transplantation

Frédéric Baron, Mohamad Mohty, Didier Blaise, Gérard Socié, Myriam Labopin, Jordi Esteve, Fabio Ciceri, Sebastian Giebel, Norbert Claude Gorin, Bipin N Savani, Christoph Schmid, Arnon Nagler



The emerging role of immune checkpoint inhibition in malignant lymphoma

Ida Hude, Stephanie Sasse, Andreas Engert, Paul J. Bröckelmann



The myeloma stem cell concept, revisited: from phenomenology to operational terms

Hans Erik Johnsen, Martin Bøgsted, Alexander Schmitz, Julie Støve Bødker, Tarec Christoffer El-Galaly, Preben Johansen, Peter Valent, Niklas Zojer, Els Van Valckenborgh, Karin Vanderkerken, Mark van Duin, Pieter Sonneveld, Martin Perez-Andres, Alberto Orfao, Karen Dybkær


‘Trained immunity’: consequences for lymphoid malignancies

Wendy B.C. Stevens, Mihai G. Netea, Arnon P. Kater, Walter J.F.M. van der Velden


New insights on hereditary erythrocyte membrane defects

Immacolata Andolfo, Roberta Russo, Antonella Gambale, Achille Iolascon


Osteonecrosis in children with acute lymphoblastic leukemia

Marina Kunstreich, Sebastian Kummer, Hans-Juergen Laws, Arndt Borkhardt, Michaela Kuhlen


Advanced systemic mastocytosis: from molecular and genetic progress to clinical practice

Celalettin Ustun, Michel Arock, Hanneke C. Kluin-Nelemans, Andreas Reiter, Wolfgang R. Sperr, Tracy George, Hans-Peter Horny, Karin Hartmann, Karl Sotlar, Gandhi Damaj, Olivier Hermine, Srdan Verstovsek, Dean D. Metcalfe, Jason Gotlib, Cem Akin, Peter Valent



Clinical impact of recurrently mutated genes on lymphoma diagnostics: state-of-the-art and beyond

Richard Rosenquist, Andreas Rosenwald, Ming-Qing Du, Gianluca Gaidano, Patricia Groenen, Andrew Wotherspoon, Paolo Ghia, Philippe Gaulard, Elias Campo, Kostas Stamatopoulos



The contribution of mouse models to the understanding of constitutional thrombocytopenia

Catherine Léon, Arnaud Dupuis, Christian Gachet, François Lanza




Bone marrow fibrosis in myelofibrosis: pathogenesis, prognosis and targeted strategies

Abdallah Abou Zahr, Mohamed E. Salama, Nicole Carreau, Douglas Tremblay, Srdan Verstovsek, Ruben Mesa, Ronald Hoffman, John Mascarenhas





Role of the tumor microenvironment in mature B-cell lymphoid malignancies

Nathan H. Fowler, Chan Yoon Cheah, Randy D. Gascoyne, John Gribben, Sattva S. Neelapu, Paolo Ghia, Catherine Bollard, Stephen Ansell, Michael Curran, Wyndham H. Wilson, Susan O’Brien, Cliona Grant, Richard Little, Thorsten Zenz, Loretta J. Nastoupil, Kieron Dunleavy


Chronic myeloid leukemia: reminiscences and dreams

Tariq I. Mughal, Jerald P. Radich, Michael W. Deininger, Jane F. Apperley, Timothy P. Hughes, Christine J. Harrison, Carlo Gambacorti-Passerini, Giuseppe Saglio, Jorge Cortes, George Q. Daley






Risk factors for relapse after allogeneic transplantation in acute myeloid leukemia

Gert J. Ossenkoppele, Jeroen J.W.M. Janssen, Arjan A. van de Loosdrecht


Humanized hemato-lymphoid system mice

Alexandre P.A. Theocharides, Anthony Rongvaux, Kristin Fritsch, Richard A. Flavell, Markus G. Manz






Environmental determinants of severity in sickle cell disease

Sanjay Tewari, Valentine Brousse, Frédéric B. Piel, Stephan Menzel, David C. Rees







Epigenetics in the hematologic malignancies

Chun Yew Fong, Jessica Morison, Mark A. Dawson


RAS diseases in children

Charlotte M. Niemeyer




Treatment of autoimmune hemolytic anemias

Alberto Zanella, Wilma Barcellini


Survival analysis in hematologic malignancies: recommendations for clinicians

Julio Delgado, Arturo Pereira, Neus Villamor, Armando López-Guillermo, Ciril Rozman


Early lymphoid lesions: conceptual, diagnostic and clinical challenges

Karthik A. Ganapathi, Stefania Pittaluga, Oreofe O. Odejide, Arnold S. Freedman, Elaine S. Jaffe


Targeting acute myeloid leukemia stem cells: a review and principles for the development of clinical trials

Daniel A. Pollyea, Jonathan A. Gutman, Lia Gore, Clayton A. Smith, Craig T. Jordan


The outcome of B-cell receptor signaling in chronic lymphocytic leukemia: proliferation or anergy

Graham Packham, Serge Krysov, Alex Allen, Natalia Savelyeva, Andrew J. Steele, Francesco Forconi, Freda K. Stevenson




Heme and erythropoieis: more than a structural role

Deborah Chiabrando, Sonia Mercurio, Emanuela Tolosano


Nature and importance of follicular lymphoma precursors

Emilie Mamessier, Florence Broussais-Guillaumot, Bruno Chetaille, Reda Bouabdallah, Luc Xerri, Elaine S. Jaffe, Bertrand Nadel


Why methylation is not a marker predictive of response to hypomethylating agents

Maria Teresa Voso, Valeria Santini, Emiliano Fabiani, Luana Fianchi, Marianna Criscuolo, Giulia Falconi, Francesco Guidi, Stefan Hohaus, Giuseppe Leone


Primary thrombocytosis in children

Nicole Kucine, Katherine M. Chastain, Michelle B. Mahler, James B. Bussel


The role of positron emission tomography-computed tomography and magnetic resonance imaging in diagnosis and follow up of multiple myeloma

Jo Caers, Nadia Withofs, Jens Hillengass, Paolo Simoni, Elena Zamagni, Roland Hustinx, Yves Beguin


Co-operating STAT5 and AKT signaling pathways in chronic myeloid leukemia and mastocytosis: possible new targets of therapy

Siham Bibi, Melis Dilara Arslanhan, Florent Langenfeld, Sylvie Jeanningros, Sabine Cerny-Reiterer, Emir Hadzijusufovic, Luba Tchertanov, Richard Moriggl, Peter Valent, Michel Arock


Management and treatment of osteonecrosis in children and adolescents with acute lymphoblastic leukemia

Mariël L. te Winkel, Rob Pieters, Ernst-Jan D. Wind, J.H.J.M. (Gert) Bessems, Marry M. van den Heuvel-Eibrink


Measurement of adherence to BCR-ABL inhibitor therapy in chronic myeloid leukemia: current situation and future challenges

Lucien Noens, Marja Hensen, Izabela Kucmin-Bemelmans, Christina Lofgren, Isabelle Gilloteau, Bernard Vrijens


Update on treatment of light chain amyloidosis

Shameem Mahmood, Giovanni Palladini, Vaishali Sanchorawala, Ashutosh Wechalekar


B-cell lymphoma mutations: improving diagnostics and enabling targeted therapies

José P. Vaqué, Nerea Martínez, Ana Batlle-López, Cristina Pérez, Santiago Montes-Moreno, Margarita Sánchez-Beato, Miguel A. Piris


Targeting the phosphoinositide 3-kinase pathway in hematologic malignancies

Elias Jabbour, Oliver G. Ottmann, Michael Deininger, Andreas Hochhaus



Advances in myelofibrosis: a clinical case approach

John O. Mascarenhas, Attilio Orazi, Kapil N. Bhalla, Richard E. Champlin, Claire Harrison, Ronald Hoffman


Autophagy in blood cancers: biological role and therapeutic implications

Alessio Nencioni, Michele Cea, Fabrizio Montecucco, Valter D. Longo, Franco Patrone, Angelo M. Carella, Tessa L. Holyoake, G. Vignir Helgason


Management recommendations for chronic myelomonocytic leukemia: consensus statements from the SIE, SIES, GITMO groups

Francesco Onida, Giovanni Barosi, Giuseppe Leone, Luca Malcovati, Enrica Morra, Valeria Santini, Giorgina Specchia, Sante Tura



Classical Hodgkin’s lymphoma: the Lymphoma Study Association guidelines for relapsed and refractory adult patients eligible for transplant

Eric Van Den Neste, Olivier Casasnovas, Marc André, Mohamed Touati, Delphine Senecal, Véronique Edeline, Aspasia Stamatoullas, Luc Fornecker, Bénédicte Deau, Thomas Gastinne, Oumédaly Reman, Isabelle Gaillard, Cécile Borel, Pauline Brice, Christophe Fermé



Indications for hematopoietic stem cell transplantation in patients with follicular lymphoma: a consensus project of the EBMT-Lymphoma Working Party

Silvia Montoto, Paolo Corradini, Martin Dreyling, Michele Ghielmini, Eva Kimby, Armando López-Guillermo, Stephen Mackinnon, Robert E. Marcus, Gilles Salles, Harry C Schouten, Anna Sureda, Peter Dreger


Non-transfusion-dependent thalassemias

Khaled M. Musallam, Stefano Rivella, Elliott Vichinsky, Eliezer A. Rachmilewitz


Iron refractory iron deficiency anemia

Luigia De Falco, Mayka Sanchez, Laura Silvestri, Caroline Kannengiesser, Martina U. Muckenthaler, Achille Iolascon, Laurent Gouya, Clara Camaschella, Carole Beaumont



Clinical implications of the molecular genetics of chronic lymphocytic leukemia

Robin Foà, Ilaria Del Giudice, Anna Guarini, Davide Rossi, Gianluca Gaidano


Diagnosis and treatment of mucormycosis in patients with hematological malignancies: guidelines from the 3rd European Conference on Infections in Leukemia (ECIL 3)

Anna Skiada, Fanny Lanternier, Andreas H. Groll, Livio Pagano, Stephan Zimmerli, Raoul Herbrecht, Olivier Lortholary, George L. Petrikkos





Infections in myelodysplastic syndromes

Andréa Toma, Pierre Fenaux, François Dreyfus, Catherine Cordonnier


Molecular pathogenesis of Waldenström’s macroglobulinemia

Esteban Braggio, Casey Philipsborn, Anne Novak, Lucy Hodge, Stephen Ansell, Rafael Fonseca




Acute myeloid leukemia developing in patients with autoimmune diseases

Safaa M. Ramadan, Tamer M Fouad, Valentina Summa, Syed KH Hasan, Francesco Lo-Coco


Thrombolytic therapy for central venous catheter occlusion

Jacquelyn L. Baskin, Ulrike Reiss, Judith A. Wilimas, Monika L. Metzger, Raul C. Ribeiro, Ching-Hon Pui, Scott C. Howard


MicroRNAs are shaping the hematopoietic landscape

Ute Bissels, Andreas Bosio, Wolfgang Wagner


Management of fertility in patients treated for Hodgkin’s lymphoma

Stephanie Harel, Christophe Fermé, Catherine Poirot



An overview of the progress on double umbilical cord blood transplantation

Anastasia Sideri, Nikolaos Neokleous, Philippe Brunet De La Grange, Bernadette Guerton, Marie-Caroline Le Bousse Kerdilles, Georges Uzan, Corina Peste-Tsilimidos, Eliane Gluckman




Chronic lymphocytic leukemia and autoimmunity: a systematic review

Kate Hodgson, Gerardo Ferrer, Emili Montserrat, Carol Moreno


The Ph-positive and Ph-negative myeloproliferative neoplasms: some topical pre-clinical and clinical issues

Richard A. Van Etten, Steffen Koschmieder, Francois Delhommeau, Danilo Perrotti, Tessa Holyoake, Animesh Pardanani, Ruben Mesa, Tony Green, Amr R. Ibrahim, Tariq Mughal, Robert Peter Gale, John Goldman


The interplay between Epstein-Barr virus and the immune system: a rationale for adoptive cell therapy of EBV-related disorders

Anna Merlo, Riccardo Turrini, Riccardo Dolcetti, Debora Martorelli, Elena Muraro, Patrizia Comoli, Antonio Rosato



PIM serine/threonine kinases in the pathogenesis and therapy of hematologic malignancies and solid cancers

Laurent Brault, Christelle Gasser, Franz Bracher, Kilian Huber, Stefan Knapp, Jürg Schwaller


Role of the PI3K/AKT and mTOR signaling pathways in acute myeloid leukemia

Sophie Park, Nicolas Chapuis, Jérôme Tamburini, Valérie Bardet, Pascale Cornillet-Lefebvre, Lise Willems, Alexa Green, Patrick Mayeux, Catherine Lacombe, Didier Bouscary



Lymphoma stem cells: enough evidence to support their existence?

Jose A. Martinez-Climent, Lorena Fontan, Randy D. Gascoyne, Reiner Siebert, Felipe Prosper


Anti-CD20 monoclonal antibodies: historical and future perspectives

Sean H. Lim, Stephen A. Beers, Ruth R. French, Peter W.M. Johnson, Martin J. Glennie, Mark S. Cragg


Genetic and immune-related factors in the pathogenesis of lymphoproliferative and plasma cell malignancies

Sigurdur Y. Kristinsson, Lynn R. Goldin, Magnus Björkholm, Jill Koshiol, Ingemar Turesson, Ola Landgren




Matriptase-2 (TMPRSS6): a proteolytic regulator of iron homeostasis

Andrew J. Ramsay, John D. Hooper, Alicia R. Folgueras, Gloria Velasco, Carlos López-Otín


Platelet receptors and signaling in the dynamics of thrombus formation

José Rivera, María Luisa Lozano, Leyre Navarro-Núñez, Vicente Vicente



Mesenchymal stem cells: the fibroblasts’ new clothes?

Muzlifah A. Haniffa, Matthew P. Collin, Christopher D. Buckley, Francesco Dazzi