Bessler M, Fehr J
Department of Medicine, University Hospital, Zurich, Switzerland.
Eur J Haematol. 1991 Sep;47(3):179-84. doi: 10.1111/j.1600-0609.1991.tb01552.x.
The FcRIII on human granulocytes is a glycosyl-phosphatidylinositol-anchored membrane protein. In PNH, proteins with this type of membrane linkage are known to be deficient on blood cells. The purpose of this study was to assess the diagnostic value of flow cytometric FcRIII quantification on granulocytes in PNH. Immunofluorescence measurements were performed in 105 patients, including 7 patients with PNH, 16 patients with aplastic anemia, and 12 with myelodysplastic syndrome, by a whole blood immunofluorescent staining procedure using monoclonal antibodies to FcRIII. In all 7 PNH patients and in 3 patients with aplastic anaemia, a distinct FcRIII-deficient granulocyte population of variable size was found. None of the remaining patients showed a similar population of FcRIII-deficient granulocytes. Quantification of FcRIII-deficient neutrophils is shown to be a highly specific and sensitive diagnostic test for PNH. It is easy, fast and highly reproducible and, therefore, suitable for routine diagnostic and follow-up studies in PNH patients and patients with aplastic anemia.
人类粒细胞上的FcRIII是一种糖基磷脂酰肌醇锚定的膜蛋白。在阵发性睡眠性血红蛋白尿症（PNH）中，已知具有这种膜连接类型的蛋白质在血细胞上缺乏。本研究的目的是评估流式细胞术对PNH患者粒细胞进行FcRIII定量的诊断价值。通过使用针对FcRIII的单克隆抗体的全血免疫荧光染色程序，对105例患者进行了免疫荧光测量，其中包括7例PNH患者、16例再生障碍性贫血患者和12例骨髓增生异常综合征患者。在所有7例PNH患者和3例再生障碍性贫血患者中，发现了不同大小的明显缺乏FcRIII的粒细胞群。其余患者均未显示出类似的缺乏FcRIII的粒细胞群。FcRIII缺陷型中性粒细胞的定量检测显示是一种对PNH高度特异且敏感的诊断试验。它简便、快速且具有高度可重复性，因此适用于PNH患者和再生障碍性贫血患者的常规诊断及随访研究。
