SYSTEMIC-DISEASES IN MYELODYSPLASTIC SYND ROMES

Myelodysplastic syndromes (MDS) are characterized by quantitative and functional involvement of myeloid lineages. Yet, systemic manifestatio ns, suggestive of a lymphocytic involvement, have been described in MD S. We review here biological and clinical data concerning the associat ions between MDS and immunological disorders. Biological auto-immune m arkers are in fact rare in MDS, and especially encountered in the chro nic myelomonocytic leukemia subgroup. Only a few systemic diseases see m to be frequently associated with MDS: seronegative arthritis, cutane ous vasculitis, and relapsing polychondritis. These diseases are proba bly not entirely auto-immune, and the signification of their associati on with an MDS remains nuclear. 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 i n men suffering from a refractory anemia (with or without excess of bl asts). The main cytogenetic abnormalities are monosomy 7, presence of a ring chromosome, and monosomy 16.