一项使用高灵敏度流式细胞术对骨髓增生异常综合征患者的阵发性睡眠性血红蛋白尿克隆进行的前瞻性横断面研究：单中心经验
A Prospective, Cross Sectional Study of PNH Clone in MDS Patients Using High Sensitivity Flowcytometry: A Single Center Experience.
作者信息
Naim Faran, Saraf Amrita, Dass Jasmita, Arya Vandana, Langer Sabina, Gupta Nitin, Sharma Ajay, Kotwal Jyoti
机构信息
Department of Clinical Hematology, Sir Ganga Ram Hospital, New Delhi, India.
Department of Hematology, Sir Ganga Ram Hospital, New Delhi, 110060 India.
出版信息
Indian J Hematol Blood Transfus. 2020 Jul;36(3):519-525. doi: 10.1007/s12288-019-01219-1. Epub 2019 Oct 24.
Subclinical PNH can be present in patients with bone marrow failure like aplastic anemia and myelodysplastic syndrome (MDS). Such clone may have prognostic and therapeutic implications. In literature around 1-10% MDS cases have shown a PNH clone, however, data from India is relatively scarce. A high sensitivity PNH assay was employed using a single tube combination of FLAER, CD157, CD64, CD15 and CD45 antibodies in adult patients of MDS at presentation. A clone size of > 0.01% was taken as significant. A total of 30 patients were included. PNH clone was present in 30% cases. Correlation done between PNH clone size and LDH values showed moderately positive correlation (r = 0.735, = 0.001, r = 0.541). As per this study a LDH cut off of 247 IU is likely to predict a PNH clone (> 1%) with moderate sensitivity and specificity. High sensitivity PNH assay is able to detect small PNH clone. Calculating the cut-off of LDH to predict PNH positivity can help us judiciously prescribe this test in MDS patients in resource constrained settings.
亚临床阵发性睡眠性血红蛋白尿（PNH）可出现在骨髓衰竭患者中，如再生障碍性贫血和骨髓增生异常综合征（MDS）。这种克隆可能具有预后和治疗意义。在文献中，约1%-10%的MDS病例显示存在PNH克隆，然而，来自印度的数据相对较少。在MDS成年患者初诊时，采用一种单管组合检测法，使用FLAER、CD157、CD64、CD15和CD45抗体进行高灵敏度PNH检测。克隆大小>0.01%被视为有意义。共纳入30例患者。30%的病例存在PNH克隆。PNH克隆大小与乳酸脱氢酶（LDH）值之间的相关性显示为中度正相关（r = 0.735，P = 0.001，r = 0.541）。根据本研究，LDH临界值为247 IU可能以中度敏感性和特异性预测PNH克隆（>1%）。高灵敏度PNH检测能够检测到小的PNH克隆。计算LDH临界值以预测PNH阳性可帮助我们在资源有限的环境中明智地为MDS患者开具此项检测。
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