Hebbar M, Hebbar-Savéan K, Fenaux P
Service de médecine interne A, CHU de Lille, France.
Rev Med Interne. 1995;16(12):897-904. doi: 10.1016/0248-8663(96)80810-1.
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.
骨髓增生异常综合征（MDS）的特征是髓系谱系的数量和功能受累。然而，MDS中已有提示淋巴细胞受累的全身表现的描述。我们在此回顾有关MDS与免疫紊乱之间关联的生物学和临床数据。事实上，生物学自身免疫标志物在MDS中很少见，尤其在慢性粒单核细胞白血病亚组中出现。似乎只有少数全身性疾病常与MDS相关：血清阴性关节炎、皮肤血管炎和复发性多软骨炎。这些疾病可能并非完全自身免疫性疾病，它们与MDS关联的意义仍不清楚。约30%的复发性多软骨炎与MDS相关。相反，0.6%的MDS与复发性多软骨炎相关。这些关联主要见于患有难治性贫血（伴或不伴原始细胞增多）的男性。主要的细胞遗传学异常为7号染色体单体、环形染色体的存在和16号染色体单体。
