Development of the diagnostic system of bone marrow failure with a minor population of PNH-phenotype cells

尽管PNH细胞的检测可作为骨髓衰竭的免疫病理生理指标，但当PNH细胞的百分比低于0.1%时，检测PNH细胞往往是困难的。我们通过对GPI锚定蛋白在流式细胞仪斑点图上的荧光强度的数学分析，建立了一种客观定量PNH型的方法，但该方法很难推广。用荧光标记的溶气素(FLARE)代替单抗，大大提高了PNH型细胞检测的准确性。然而，一些没有PNH类型细胞增加的患者对免疫抑制治疗有反应。为了找出一个更好的骨髓衰竭免疫病理生理学标志物，我们研究了血浆血小板生成素(TPO)水平与骨髓衰竭病理生理学的相关性。TPO水平为320 pg/ml的骨髓衰竭患者被证实患有免疫病理生理学良性疾病，实际上包括所有PNH类型细胞增加的患者。因此，测定血浆TPO水平有助于诊断骨髓衰竭的病理生理学。

Although detection of PNH-type cells is useful as a marker for immune pathophysiology of bone marrow failure, it is often difficult when the percentage of PNH-type cells is less than 0.1%. We established an objective method of quantifying PNH-type with mathematical analysis of the fluorescent intensity of GPI-anchored proteindots on the flow cytometry dotgram, but the method was difficult to generalize. The use of fluorescent labeled aerolysin (FLARE) instead of monoclonal antibodies greatly improved the accuracy of detecting PNH-type cells. However, some patients without increased PNH-type cells respond to immunosuppressive therapy. To identify a better marker for immune pathophysiology of bone marrow failure, we examined a correlation of plasma thrombopoietin (TPO) levels with pathophysiology of bone marrow failure. Bone marrow failure patients showing TPO levels>320 pg/ml proved to have benign diseases with immune pathophysiology and virtually included all patients with increased PNH-type cells. The measurement of plasma TPO levels is therefore useful for diagnosing pathophysiology of bone marrow failure.