Noji H, Shichishima T, Ishikawa S, Kai T, Saitoh Y, Maruyama Y
First Department of Internal Medicine, Fukushima Medical University.
Rinsho Ketsueki. 1999 Mar;40(3):240-3.
A 25-year-old man was admitted for evaluation of pancytopenia on May 2, 1997. On admission, he had pancytopenia with a normal reticulocyte count. Bone marrow aspirate specimens displayed a normal karyotype and hypocellularity without myelodysplasia. Although total bilirubin and lactate dehydrogenase levels were within their normal ranges, the haptoglobin level was low; additionally, two-color flow cytometric analysis determined that 3.3% of erythrocytes were double-negative for CD55 and CD59 expression. Atypical paroxysmal nocturnal hemoglobinuria with bone marrow hypoplasia was diagnosed. Because initial treatment with cyclosporin A was not effective, the patient was subsequently given a combination of antithymocyte globulin, cyclosporin A, and granulocyte colony-stimulating factor. Although the pancytopenia subsided, the percentage of double-negative erythrocytes in the patient's blood remained almost unchanged compared to findings obtained on admission.
一名25岁男性于1997年5月2日因全血细胞减少症入院接受评估。入院时，他全血细胞减少，网织红细胞计数正常。骨髓穿刺标本显示核型正常且细胞减少，无骨髓发育异常。尽管总胆红素和乳酸脱氢酶水平在正常范围内，但触珠蛋白水平较低；此外，双色流式细胞术分析确定3.3%的红细胞CD55和CD59表达呈双阴性。诊断为伴有骨髓发育不全的非典型阵发性睡眠性血红蛋白尿症。由于最初使用环孢素A治疗无效，该患者随后接受了抗胸腺细胞球蛋白、环孢素A和粒细胞集落刺激因子的联合治疗。尽管全血细胞减少症有所缓解，但与入院时的检查结果相比，患者血液中双阴性红细胞的百分比几乎没有变化。
