Jin J Y, Tooze J A, Marsh J C, Matthey F, Gordon-Smith E C
Department of Cellular and Molecular Sciences, St George's Hospital Medical School, London.
Br J Haematol. 1996 Sep;94(3):510-2. doi: 10.1046/j.1365-2141.1996.d01-1831.x.
Myelodysplasia (MDS) and aplastic anaemia-paroxysmal nocturnal haemoglobinuria (AA/PNH) syndrome developed in a severe aplastic anaemia (AA) patient after treatment with immunosuppressive (IS) therapy. Glycosylphosphatidyl inositol (GPI)-linked proteins were determined, and during the AA/PNH phase, a high proportion of neutrophils were found to be negative, without clinical evidence of haemolysis. However, MDS developed with cytogenetic abnormalities of monosomy 7,9q- and a rearranged chromosome 6; the GPI-linked protein negative cells were completely replaced by positively expressing cells. This represents the emergence of a GPI-linked protein positive myelodysplasia clone arising separately from an AA/PNH clone.
一名重型再生障碍性贫血（AA）患者在接受免疫抑制（IS）治疗后发生了骨髓增生异常综合征（MDS）和再生障碍性贫血-阵发性睡眠性血红蛋白尿（AA/PNH）综合征。对糖基磷脂酰肌醇（GPI）连接蛋白进行了检测，在AA/PNH阶段，发现高比例的中性粒细胞呈阴性，且无溶血的临床证据。然而，MDS出现了7号染色体单体、9q-和6号染色体重排的细胞遗传学异常；GPI连接蛋白阴性细胞被阳性表达细胞完全取代。这代表了一个与AA/PNH克隆分开产生的GPI连接蛋白阳性骨髓增生异常克隆的出现。
