• T Comont, K Delavigne, P Cougoul, S Bertoli, E Delabesse, P Fenaux, and O Beyne-Rauzy.
• Service de médecine interne et immunopathologie, institut universitaire du Cancer de Toulouse Oncopôle, centre hospitalier universitaire de Toulouse, 31100 Toulouse, France; UFR Purpan, université Toulouse III Paul Sabatier, 31400 Toulouse, France; UMR1037-Inserm, ERL5294 CNRS, centre de recherche en cancérologie de Toulouse, 31100 Toulouse, France. Electronic address: comont.thibault@iuct-oncopole.fr.
• Rev Med Interne. 2019 Sep 1; 40 (9): 581-589.

AbstractMyelodysplastic syndromes are a heterogeneous group of clonal myeloid disorders characterized by peripheral cytopenias and an increased risk of progression to acute myeloid leukemia. Inflammatory, auto-immune or syndromic symptoms can make the diagnosis difficult. Diagnosis is currently based on bone marrow cytology but cytogenetics and molecular features are currently overpassing their initial prognostic function (allowing early diagnosis and prediction of therapeutic response). The prognostic classification is based on the Revised International Prognostic Scoring System, which also provides guidance for therapeutic management. The treatment of low-risk myelodysplastic syndromes is based on the correction of cytopenias (erythropoiesis stimulating agents, transfusions, lenalidomide, etc.), whereas in high-risk group, the goal is the control of the leukemic clone (hypomethylating agents, allograft of hematopoietic stem cell transplantation). Other molecules are used to manage complications of cytopenias or transfusion (anti-infectious prophylaxis and treatments, martial chelation). New molecules are being studied with some interesting results (luspatercept, venetoclax). This article aims to provide an update on the knowledge that an internist should know for the practical management of myelodysplastic syndromes in 2019.Copyright © 2019 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

12 keywords...

hide…