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

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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.