[Dysimmune manifestations associated with myelodysplastic neoplasms and chronic myelomonocytic leukaemias].

Item request has been placed! ×
Item request cannot be made. ×
loading   Processing Request
Read More Add to Saved list
  • Additional Information
    • Transliterated Title:
      Manifestations dysimmunitaires associées aux syndromes myélodysplasiques et leucémies myélomonocytaires chroniques.
    • Source:
      Publisher: Elsevier Country of Publication: France NLM ID: 0072416 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1769-6917 (Electronic) Linking ISSN: 00074551 NLM ISO Abbreviation: Bull Cancer Subsets: MEDLINE
    • Publication Information:
      Publication: Jan. 2015- : Paris : Elsevier
      Original Publication: Paris : Masson
    • Subject Terms:
      Leukemia, Myelomonocytic, Chronic*/complications ; Leukemia, Myelomonocytic, Chronic*/genetics ; Leukemia, Myelomonocytic, Chronic*/drug therapy ; Myelodysplastic Syndromes*/complications ; Myelodysplastic Syndromes*/genetics ; Skin Diseases*; Humans ; Azacitidine/therapeutic use
    • Abstract:
      Systemic inflammatory or autoimmune diseases (SIAD) are observed in up to a quarter of patients with myelodysplastic syndromes (MDS) or chronic myelomonocytic leukemia (CMML), with a broad clinical spectrum including asymptomatic biological abnormalities, isolated inflammatory clinical manifestations (recurrent fever, arthralgia, neutrophilic dermatoses…) or identified systemic diseases (giant cell arteritis, recurrent polychondritis…). Recent advances in molecular biology have shed new light on the pathophysiological mechanisms that link inflammatory manifestations and myeloid hemopathies, particularly in VEXAS syndrome following the identification of somatic mutations in the UBA1 gene, or in neutrophilic dermatoses with the concept of myelodysplasia cutis. Although the presence of SIAD does not seem to affect overall survival or the risk of transformation into acute myeloid leukemia, their treatment remains a challenge given the frequent high level of corticosteroid dependence as well as the poor efficacy and tolerance (cytopenias, infections) of conventional immunosuppressive agents. Recent prospective data supports the interest of a therapeutic strategy using demethylating agents and notably azacitidine to target the pathological clone.
      (Copyright © 2023 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)
    • Contributed Indexing:
      Keywords: Autoimmune disease; Azacitidine; Chronic myelomonocytic leukemia; Inflammatory diseases; Leucémie myélomonocytaire chronique; Maladie auto-immune; Maladies inflammatoires; Myelodysplastic syndrome; Syndrome myélodysplasique; VEXAS syndrome
    • Accession Number:
      M801H13NRU (Azacitidine)
    • Subject Terms:
      VEXAS syndrome
    • Publication Date:
      Date Created: 20230706 Date Completed: 20231113 Latest Revision: 20231122
    • Publication Date:
      20231123
    • Accession Number:
      10.1016/j.bulcan.2023.02.023
    • Accession Number:
      37414632