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Association between systemic diseases and myelodysplasia


Hématologie. Volume 4, Number 1, 51-5, Janvier-Février 1998, Mini-revues et revues

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Author(s) : Mohamed Hebbar, Katia Hebbar-Savéan, Pierre Fenaux

Summary : Several systemic diseases have been described in association with myelodysplastic syndromes (MDS). Yet, most of these systemic diseases are frequent in the general population and their association with MDS could be fortuitous. Auto-immune diseases ob not really seem to be more frequent in patients with MDS (except CMML subtype). A link with MDS may be proposed for relapsing polychondritis, seronegative arthritis, and cutaneous vasculitis, and in these cases MDS is generally not a CMML. All these three syndromes could correspond to a same disease, since seronegative arthritis and cutaneous vasculitis are common in relapsing polychondritis. About 10 % of patients with relapsing polychondritis also have MDS, and 0,6 % of patients with MDS also have relapsing polychondritis. Pathogenesis of the association between MDS and relapsing polychondritis remains unknown. This association in mainly observed in male patients, and the most frequent cytogenetic abnormalities are monosomy 7, ring chromosome and monosomy 16.

Keywords : myelodysplastic syndromes, relapsing polychondritis, vasculitis, seronegative arthritis.

 

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