John Libbey Eurotext

Hématologie

MENU

Dysimmune manifestations of myelodysplastic syndromes and chronic myelomonocytic leukaemias Volume 28, issue 3, 2022-05-03

Figures

  • Figure 1.
  • Figure 2.

Tables

Authors
Service d’hématologie séniors, hôpital Saint-Louis, Paris, France
Service de médecine interne, hôpital Saint-Antoine, Paris, France
Service d’hématologie séniors, hôpital Saint-Louis, Paris, France
Tirés à part : LP Zhao
Liens d’intérêt : aucun

Around 25% of patients with myelodysplastic syndromes (MDS) and chronic myelomonocytic leukemia (CMML) also present systemic inflammatory and auto-immune diseases (SIAD), including inflammatory arthritis, connective tissue diseases, auto-immune cytopenias, systemic vasculitis and inflammatory cutaneous manifestations. A recent study showed a higher prevalence of TET2/IDH and SRSF2 mutations in MDS/CMML patients with SIAD compared to MDS/ CMML patients without SIAD, suggesting that these mutations may play a role in a common pathogenesis. Moreover, a “genotype-first” screening method led to the recent discovery of a new monogenic inflammatory disorder, the VEXAS syndrome (MIM #301054), further refining the heterogenous spectrum of inflammatory diseases associated with MDS/ CMML. The management of these patients remains challenging, as the use of immunosuppressive therapies is limited by the preexisting cytopenias and the susceptibility to infections. In this context, the results of azacitidine seems promising, acting on both the hematological and the inflammatory disorders.