Nakakuma H, Nagakura S, Iwamoto N, Kawaguchi T, Hidaka M, Horikawa K, Kagimoto T, Shido T, Takatsuki K
Second Department of Internal Medicine, Kumamoto University School of Medicine, College of Medical Science, Japan.
Blood. 1995 Mar 1;85(5):1371-6.
The lack of glycosylphosphatidylinositol (GPI)-anchored membrane proteins such as decay-accelerating factor (DAF) and CD59 on blood cells has a diagnostic value in paroxysmal nocturnal hemoglobinuria (PNH). Because PNH often develops in patients with aplastic anemia (AA), we attempted to detect a PNH clone in the bone marrow (BM) of patients with AA and pancytopenia before affected cells were evident in the peripheral blood (PB). We used flow cytometry with monoclonal antibodies against DAF and CD59 for the detection of the clone. Affected cells were observed in the BM of 3 of 7 patients with AA and 1 of 3 patients with pancytopenia of unknown origin, but not in their PB. All 8 patients with apparent PNH had affected cells in their BM and PB. On the basis of the early appearance of the PNH clone in the BM, a prospective 4-month follow-up study of the PB cells was performed. The study showed the release of affected mature cells first in granulocytes, then in monocytes, and finally in lymphocytes. Ham's test was positive before affected erythrocytes were detected by flow cytometry. Our findings indicate that detection of the PNH clone in BM could be predictive of the development of PNH in patients with AA and pancytopenia.
血细胞上缺乏糖基磷脂酰肌醇（GPI）锚定膜蛋白，如衰变加速因子（DAF）和CD59，在阵发性夜间血红蛋白尿（PNH）中具有诊断价值。由于PNH常发生于再生障碍性贫血（AA）患者，我们试图在血细胞减少的AA患者骨髓（BM）中检测PNH克隆，此时外周血（PB）中受影响的细胞尚不明显。我们使用针对DAF和CD59的单克隆抗体通过流式细胞术检测该克隆。在7例AA患者中的3例以及3例不明原因血细胞减少患者中的1例的骨髓中观察到了受影响的细胞，但在外周血中未观察到。所有8例明显的PNH患者的骨髓和外周血中均有受影响的细胞。基于PNH克隆在骨髓中的早期出现，对其外周血细胞进行了为期4个月的前瞻性随访研究。该研究表明，首先在粒细胞中释放受影响的成熟细胞，然后是单核细胞，最后是淋巴细胞。在通过流式细胞术检测到受影响的红细胞之前，Ham试验呈阳性。我们的研究结果表明，在骨髓中检测PNH克隆可以预测AA和血细胞减少患者中PNH的发展。
