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KMID : 1100720130330040288
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Annals of Laboratory Medicine
2013 Volume.33 No. 4 p.288 ~ p.292
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Rapid Determination of Chimerism Status Using Dihydrorhodamine Assay in a Patient with X-linked Chronic Granulomatous Disease Following Hematopoietic Stem Cell Transplantation
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Kim Hyun-Young
Kim Hee-Jin
Ki Chang-Seok
Kim Dae-Won
Kang Eun-Suk
Yoo Keon-Hee
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Abstract
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Chronic granulomatous disease (CGD) is a rare genetic disease, which is caused by defects in the NADPH oxidase complex (gp91phox, p22phox, p40phox, p47phox, and p67phox) of phagocytes. This defect results in impaired production of superoxide anions and other reactive oxygen species (ROS), which are necessary for killing bacterial and fungal microorganisms and leads to recurrent, life-threatening bacterial and fungal infections and granulomatous inflammation. The dihydrorhodamine (DHR) flow cytometry assay is a useful diagnostic tool for CGD that can detect absent or reduced NADPH oxidase activity in stimulated phagocytes. We report a patient with X-linked CGD carrying a novel mutation of the CYBB gene whose chimerism status following hematopoietic stem cell transplantation (HSCT) has been rapidly determined using the DHR assay. The level of DHR activity correlates well with short tandem repeat PCR analysis. Considering the advantages of this simple, rapid, and cost-effective procedure, serial measurement of DHR assay would facilitate the rapid determination of a patient’s engraftment status, as a supplementary monitoring tool of chimerism status following HSCT.
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KEYWORD
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Chronic granulomatous disease, Dihydrorhodamine assay, Chimerism
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