Luraschi A, Saglietti G, Fedeli P, Gioria A, Della Vedova A, Ferrari V, Borgotti P
Divisione di Medicina, USSL n. 57 Piemonte, Ospedale di Omegna, Novara.
Minerva Med. 1994 Apr;85(4):145-53.
Several studies aiming to describe the immunological abnormalities occurring in patients affected by myelodysplastic syndromes (MDS) have been carried out in recent years. We report on the immunological abnormalities found in 22 myelodysplastic patients at the time of diagnosis (RA: 2 cases; ASIA: 4 cases; AREB: 6 cases; AREB-T: 6 cases. LMMC: 4 cases). Matched with similarly aged healthy people (controls) all our patients revealed a significant lymphocytopenia mainly due to a reduction both in number and percentage of T-helper series with decreased OK T4/OK T8 ratio as a result; even B-cells were reduced in number but their percentage still overlapped with the controls. Out of 22 patients, 13 showed hypergammaglobulinemia (polyclonal in 12 cases, monoclonal in the one left) and 2 read positive for Coomb's and Ana-test respectively. The involvement of T-cell immunity in the course of MDS can be explained if we consider the clonal origin of such diseases. Among myelodysplastic patients the ones affected by LMMC, AREB and AREB-T show the heaviest immunological abnormalities: in these cases the whole of T-cells subsets and NK cells as well are affected. Eventually, the mentioned abnormalities are of paramount importance to explain how easily these patients can develop both severe infectious diseases and abrupt acute leukemia.
近年来，已经开展了多项旨在描述骨髓增生异常综合征（MDS）患者所出现的免疫异常的研究。我们报告了22例骨髓增生异常患者在诊断时所发现的免疫异常情况（难治性贫血：2例；难治性贫血伴环形铁粒幼细胞增多：4例；难治性贫血伴原始细胞增多：6例；难治性贫血伴原始细胞增多-转变型：6例；低增生性骨髓增生异常综合征：4例）。与年龄相仿的健康人（对照组）相比，我们所有的患者均出现显著的淋巴细胞减少，主要是由于辅助性T细胞系列的数量和百分比均下降，结果OK T4/OK T8比值降低；甚至B细胞数量也减少，但其百分比仍与对照组重叠。在22例患者中，13例表现为高球蛋白血症（12例为多克隆性，1例为单克隆性），2例分别抗人球蛋白试验和ANA试验呈阳性。如果我们考虑此类疾病的克隆起源，就可以解释T细胞免疫在MDS病程中的参与情况。在骨髓增生异常患者中，受低增生性骨髓增生异常综合征、难治性贫血伴原始细胞增多和难治性贫血伴原始细胞增多-转变型影响的患者表现出最严重的免疫异常：在这些病例中整个T细胞亚群以及NK细胞也都受到影响。最终，上述异常对于解释这些患者为何容易发生严重感染性疾病和急性白血病至关重要。
