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Normal and pathological erythropoiesis
in adults: from gene regulation to targeted treatment concepts
Peter Valent,1,2 Guntram Büsche,3 Igor Theurl,4 Iris Z. Uras,5 Ulrich Germing,6 Reinhard Stauder,7 Karl Sotlar,8 Wolfgang Füreder,1 Peter Bettelheim,9 Michael Pfeilstöcker,2,10 Rainer Oberbauer,11 Wolfgang R. Sperr,1,2 Klaus Geissler,12 Jürg Schwaller,13 Richard Moriggl,14,15 Marie C. Béné,16 Ulrich Jäger,1,2 Hans-Peter Horny17 and Olivier Hermine18
1Department of Internal Medicine I, Division of Hematology & Hemostaseology, Medical University of Vienna, Austria; 2Ludwig Boltzmann Cluster Oncology, Medical University of Vienna, Austria; 3Institute of Pathology, Medizinische Hochschule Hannover, Germany; 4Department of Internal Medicine II, Medical University Innsbruck, Austria; 5Institute of Pharmacology and Toxicology, University of Veterinary Medicine, Vienna, Austria; 6Department of Hematology, Oncology and Clinical Immunology, Heinrich-Heine University, Düsseldorf, Germany; 7Department of Internal Medicine V, Medical University Innsbruck, Austria; 8Institute of Pathology, Paracelsus Medical University Salzburg, Austria; 9First Department of Internal Medicine, Elisabethinen Hospital, Linz, Austria; 103rd Medical Department, Hanusch Hospital, Vienna, Austria; 11Department of Nephrology and Dialysis, Medical University of Vienna, Austria; 125th Medical Department for Hematology and Oncology, Hospital Hietzing, Vienna, Austria; 13Department of Biomedicine, University Children's Hospital Basel, Switzerland; 14Ludwig Boltzmann Institute for Cancer Research, Vienna, Austria; 15Department of Biomedical Science, Institute of Animal Breeding and Genetics, University of Veterinary Medicine, Vienna, Austria; 16Hematology Biology, University Hospital, Nantes, France; 17Institute of Pathology, Ludwig-Maximilian University, Munich, Germany and 18Imagine Institute, INSERM U 1163, CNRS 8654, Université Paris Descartes, Sorbonne, Paris Cité, France
ABSTRACT
Pathological erythropoiesis with consequent anemia is a leading cause of symptomatic morbidity in internal medicine. The etiologies of anemia are complex and include reactive as well as neoplastic conditions. Clonal expansion of erythroid cells in the bone marrow may result in peripheral erythrocytosis and polycythemia but can also result in anemia when clonal cells are dysplastic and have a maturation arrest that leads to apoptosis and hinders migration, a constellation typically seen in the myelodysplastic syndromes. Rarely, clonal expansion of immature erythroid blasts results in a clinical picture resembling erythroid leukemia. Although several mechanisms underlying normal and abnor- mal erythropoiesis and the pathogenesis of related disorders have been deciphered in recent years, little is known about specific markers and tar- gets through which prognosis and therapy could be improved in anemic or polycythemic patients. In order to discuss new markers, targets and novel therapeutic approaches in erythroid disorders and the related pathologies, a workshop was organized in Vienna in April 2017. The out- comes of this workshop are summarized in this review, which includes a discussion of new diagnostic and prognostic markers, the updated WHO classification, and an overview of new drugs used to stimulate or to interfere with erythropoiesis in various neoplastic and reactive condi- tions. The use and usefulness of established and novel erythropoiesis- stimulating agents for various indications, including myelodysplastic syn- dromes and other neoplasms, are also discussed.
Ferrata Storti Foundation
Haematologica 2018 Volume 103(10):1593-1603
Correspondence:
peter.valent@meduniwien.ac.at or ohermine@gmail.com
Received: March 6, 2018. Accepted: May 30, 2018. Pre-published: August 3, 2018.
doi:10.3324/haematol.2018.192518
Check the online version for the most updated information on this article, online supplements, and information on authorship & disclosures: www.haematologica.org/content/103/10/1593
©2018 Ferrata Storti Foundation
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