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Prepublished online as a Blood First Edition Paper on April 30, 2002; DOI 10.1182/blood-2002-01-0211. This Article Full Text (PDF) All Versions of this Article: 2002-01-0211v1 100/4/1208    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Yamaguchi, K. Articles by Sakiyama, Y. Search for Related Content PubMed PubMed Citation Articles by Yamaguchi, K. Articles by Sakiyama, Y. Social Bookmarking                   What's this? Submitted January 25, 2002 Accepted April 8, 2002 Mixed chimera status of 12 patients with Wiskott-Aldrich syndrome (WAS) after hematopoietic stem cell transplantation; evaluation by flow cytometric analysis of intracellular WAS protein expression Koji Yamaguchi, Tadashi Ariga*, Masafumi Yamada, David L Nelson, Ryouji Kobayashi, Chie Kobayashi, Yasushi Noguchi, Yasuhiko Ito, Kenji Katamura, Yoshihisa Nagatoshi, Satoshi Kondo, Hiroyuki Katoh, and Yukio Sakiyama Research Group of Human Gene Therapy, Hokkaido University, Graduate School of Medicine, Sapporo, Hokkaido, Japan; Department of Surgical Oncology, Hokkaido University, Graduate School of Medicine, Sapporo, Hokkaido, Japan Research Group of Human Gene Therapy, Hokkaido University, Graduate School of Medicine, Sapporo, Hokkaido, Japan Department of Pediatrics, Hokkaido University, Graduate School of Medicine, Sapporo, Hokkaido, Japan The Metabolism Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA Department of Pediatrics, Ibaraki Children's Hospital, Mito, Ibaraki, Japan Department of Pediatrics, Chiba University, Graduate School of Medicine, Chiba, Chiba, Japan Department of Pediatrics, Medical School, Nagoya City University, Nagoya, Aichi, Japan Department of Pediatrics, Kyoto University, Graduate School of Medicine, Kyoto, Kyoto, Japan Section of Pediatrics, National Kyusyu Cancer Center, Fukuoka, Fukuoka, Japan Department of Surgical Oncology, Hokkaido University, Graduate School of Medicine, Sapporo, Hokkaido, Japan * Corresponding author; email: tada-ari{at}med.hokudai.ac.jp. The Wiskott-Aldrich syndrome (WAS) is caused by defects in the WAS protein (WASP) gene on the X-chromosome. Previously we reported that flow cytometric analysis of intracellular WASP expression (FCM-WASP) was useful for the diagnosis of WAS patients and carriers. In this study, we applied FCM-WASP to evaluate the mixed chimera (MC) status of 12 WAS patients who underwent hematopoietic stem cell transplantation (HST). After HST, donor and recipient-derived peripheral blood mononuclear cells (PBMC) could be easily distinguished by this method, as the donor cells are observed to be WASPbright, while the defective recipient cells are WASPdim. Furthermore, by two-color FCM-WASP, the MC status could be characterized by cell lineage. Six of 12 WAS patients were revealed to have the MC status after HST, while others had the complete chimera status. The MC was observed in every cell lineage examined. However, among PBMC, recipient cells were most commonly observed in the monocyte population. Finally, to investigate the "naive/memory" status of donor and recipient T cells in these patients, three-color FCM-WASP using anti-CD45RA or CD45RO was performed. It was demonstrated that, in contrast to WASPbright T cells, most WASPdim T cells remained "naive" (CD45RA+/RO-) more than one year after HST. No imbalance in the ratio of "naive/memory" T cells was observed in WAS patients before HST. We conclude that FCM-WASP is a potentially useful method for clinical follow-up of WAS patients who underwent HST. This study may also have significant implications regarding the role of WASP during hematopoietic development. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: M. Bosticardo, F. Marangoni, A. Aiuti, A. Villa, and M. Grazia Roncarolo Recent advances in understanding the pathophysiology of Wiskott-Aldrich syndrome Blood, June 18, 2009; 113(25): 6288 - 6295. [Abstract] [Full Text] [PDF] S. Trifari, G. 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