[Systemic diseases in myelodysplastic syndromes].
Myelodysplastic syndromes (MDS) are characterized by quantitative and functional involvement of myeloid lineages. Yet, systemic manifestations, suggestive of a lymphocytic involvement, have been described in MDS. We review here biological and clinical data concerning the associations between MDS and immunological disorders. Biological auto-immune markers are in fact rare in MDS, and especially encountered in the chronic myelomonocytic leukemia subgroup. Only a few systemic diseases seem to be frequently associated with MDS: seronegative arthritis, cutaneous vasculitis, and relapsing polychondritis. These diseases are probably not entirely auto-immune, and the signification of their association with an MDS remains unclear. About 30% of relapsing polychondritis are associated with MDS. Conversely, 0.6% of MDS are associated with a relapsing polychondritis. These associations are mainly encountered in men suffering from a refractory anemia (with or without excess of blasts). The main cytogenetic abnormalities are monosomy 7, presence of a ring chromosome, and monosomy 16.
Hebbar
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Service de médecine interne A, CHU de Lille, France.
Hebbar-Savéan
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Fenaux
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D001168:Arthritis / Q000209:etiology; D005260:Female; D006801:Humans; D008297:Male; D009190:Myelodysplastic Syndromes / Q000150:complications* / Q000276:immunology; D011081:Polychondritis, Relapsing / Q000209:etiology; D014657:Vasculitis / Q000209:etiology
Maladies systémiques au cours des syndromes myélodysplasiques.