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[Systemic diseases in myelodysplastic syndromes]

M Hebbar1, K Hebbar-Savéan, P Fenaux

  • 1Service de médecine interne A, CHU de Lille, France.

La Revue De Medecine Interne
|January 1, 1995
PubMed
Summary

Myelodysplastic syndromes (MDS) can present with systemic symptoms suggesting immune system involvement. This review explores the links between MDS and immunological disorders, noting associations with specific conditions like relapsing polychondritis.

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Leukemia & lymphoma·2020

Area of Science:

  • Hematology
  • Immunology
  • Oncology

Context:

  • Myelodysplastic syndromes (MDS) primarily affect myeloid lineages but can exhibit systemic manifestations.
  • The association between MDS and immunological disorders requires further investigation.
  • Chronic myelomonocytic leukemia (CMML) subgroup shows a higher prevalence of biological autoimmune markers.

Purpose:

  • To review biological and clinical data on the associations between MDS and immunological disorders.
  • To clarify the significance of systemic manifestations in MDS patients.
  • To identify specific systemic diseases frequently associated with MDS.

Summary:

  • Biological autoimmune markers are infrequent in MDS, except in CMML.
  • Systemic diseases like seronegative arthritis, cutaneous vasculitis, and relapsing polychondritis are associated with MDS.
  • Relapsing polychondritis occurs in approximately 30% of MDS cases and 0.6% of relapsing polychondritis cases, predominantly in men with refractory anemia and specific cytogenetic abnormalities (monosomy 7, ring chromosome, monosomy 16).

Impact:

  • Highlights the complex relationship between hematological malignancies and immune system dysfunction.
  • Provides insights into potential diagnostic and therapeutic strategies for MDS patients with systemic manifestations.
  • Emphasizes the importance of considering MDS in patients with associated immunological conditions, particularly relapsing polychondritis.

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