Hanukoglu A, Meytes D, Fried A, Rosen N, Shacked N
Acta Paediatr Scand. 1987 May;76(3):539-43. doi: 10.1111/j.1651-2227.1987.tb10515.x.
An infant with Down's syndrome developed severe persistent neutropenia at the age of 9 months and fluctuating anemia and thrombocytopenia at one year of age which terminated as full-blown aplastic anemia at 26 months of age. Immunological evaluation revealed increased peripheral and bone marrow lymphocytes and impaired blood OKT4: OKT8 ratio. Bone marrow granulocyte-macrophage colony forming cells (GM-CFC) were markedly increased, while peripheral blood mononuclear cells (PBMN) produced normal numbers of colonies. The patient's PBMN and serum were both somewhat inhibitory to normal bone marrow derived GM-CFC, suggesting the existence of a suppressor activity both in his serum and PBMN. This unusual course of aplastic anemia and the abnormalities in T-cells and hematopoiesis in Down's syndrome are discussed.
一名患有唐氏综合征的婴儿在9个月大时出现严重持续性中性粒细胞减少，1岁时出现波动性贫血和血小板减少，并在26个月大时发展为典型的再生障碍性贫血。免疫评估显示外周血和骨髓淋巴细胞增多，血液中OKT4:OKT8比值受损。骨髓粒细胞-巨噬细胞集落形成细胞（GM-CFC）明显增加，而外周血单个核细胞（PBMN）产生的集落数量正常。患者的PBMN和血清对正常骨髓来源的GM-CFC均有一定程度的抑制作用，提示其血清和PBMN中均存在抑制活性。本文讨论了唐氏综合征患者再生障碍性贫血的这一不寻常病程以及T细胞和造血异常情况。
