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

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 abnormal erythropoiesis and the pathogenesis of related disorders have been deciphered in recent years, little is known about specific markers and targets 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 outcomes 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 conditions. The use and usefulness of established and novel erythropoiesis-stimulating agents for various indications, including myelodysplastic syndromes and other neoplasms, are also discussed.

journal_name

Haematologica

journal_title

Haematologica

authors

Valent P,Büsche G,Theurl I,Uras IZ,Germing U,Stauder R,Sotlar K,Füreder W,Bettelheim P,Pfeilstöcker M,Oberbauer R,Sperr WR,Geissler K,Schwaller J,Moriggl R,Béné MC,Jäger U,Horny HP,Hermine O

doi

10.3324/haematol.2018.192518

subject

Has Abstract

pub_date

2018-10-01 00:00:00

pages

1593-1603

issue

10

eissn

0390-6078

issn

1592-8721

pii

haematol.2018.192518

journal_volume

103

pub_type

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