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本文报道了1例 Fanconi 贫血患儿染色体和免疫学实验结果。结果表明,该患儿染色体核型为亚二倍体,畸变率为38％,形式有染色体和染色单体,裂隙,断裂,双着丝点染色体,核内复制,四射体等形式;患儿外周血单个核细胞(PBMC)中白细胞介素-2受体(IL—2R)阳性细胞明显高于普通再生障碍性贫血患儿和正常成人两个对照组;患儿抑制性 T 细胞(Ts)功能及淋巴细胞亚群也出现明显异常;患儿血清能明显抑制正常人骨髓粒—单置造血祖细胞(CFU—GM)的生长,抗人干扰素—γ(IFN—γ)单克隆抗体能部分拮抗这种抑制效应。上述结果提示,免疫学异常在 Ranconi 贫血发病中可能起重要作用.
This article reports the chromosomal and immunological results of a case of Fanconi anemia in children. The results showed that the karyotype of this child was sub-diploid with the aberration rate of 38%. The forms were chromosomal and chromatid, fissure, rupture, dicentric chromosome, nucleus duplication, Interleukin-2 receptor (IL-2R) positive cells in peripheral blood mononuclear cells (PBMC) were significantly higher than those in normal children with aplastic anemia and normal controls. The levels of inhibitory T cells (Ts ) Function and lymphocyte subsets also showed obvious abnormalities. Serum from children significantly inhibited the growth of normal human bone marrow granulocyte-monocyte-derived hematopoietic progenitor cells (CFU-GM). The anti-human interferon-γ Can partially antagonize this inhibitory effect. The above results suggest that immunological abnormalities may play an important role in the pathogenesis of Ranconi anemia.