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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 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 Citation Map 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. Related Collections Hemostasis, Thrombosis, and Vascular Biology Transplantation Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  Blood, 15 August 2002, Vol. 100, No. 4, pp. 1208-1214 CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS 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 From the Research Group of Human Gene Therapy, the Division of Cancer Medicine, Department of Surgical Oncology, and the Department of Pediatrics, Hokkaido University, Graduate School of Medicine, Sapporo, Japan; the Metabolism Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD; the Department of Pediatrics, Ibaraki Children's Hospital, Mito, Japan; the Department of Pediatrics, Chiba University, Graduate School of Medicine, Chiba, Japan; the Department of Pediatrics, Medical School, Nagoya City University, Nagoya, Japan; the Department of Pediatrics, Kyoto University, Graduate School of Medicine, Kyoto, Japan; and the Section of Pediatrics, National Kyusyu Cancer Center, Fukuoka, Japan. Wiskott-Aldrich syndrome (WAS) is caused by defects in the WAS protein (WASP) gene on the X chromosome. We previously reported that flow cytometric analysis of intracellular WASP expression (FCM-WASP) was useful in the diagnosis of WAS in 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 (PBMCs) could be distinguished easily with this method, since the donor cells were WASPbright, whereas the defective recipient cells were WASPdim. Furthermore, with use of 2-color FCM-WASP, the MC status could be characterized by cell lineage. Six of the 12 patients with WAS were found to have MC status after HST, whereas others had complete chimera status. MC status was observed in every cell lineage examined. However, among PBMCs, 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, 3-color FCM-WASP using anti-CD45RA or CD45RO was performed. We found that, in contrast to WASPbright T cells, most WASPdim T cells remained naive (CD45RA+/RO) more than a year after HST. No imbalance in the ratio of naive to 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 have undergone HST. Our findings may also have important implications for the role of WASP during hematopoietic development. © 2002 by The American Society of Hematology.   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. Sitia, A. Aiuti, S. Scaramuzza, F. Marangoni, L. G. Guidotti, S. Martino, P. Saracco, L. D. Notarangelo, M.-G. Roncarolo, et al. Defective Th1 Cytokine Gene Transcription in CD4+ and CD8+ T Cells from Wiskott-Aldrich Syndrome Patients J. Immunol., November 15, 2006; 177(10): 7451 - 7461. [Abstract] [Full Text] [PDF] T. Wada, S. H. Schurman, G. J. Jagadeesh, E. K. Garabedian, D. L. Nelson, and F. Candotti Multiple patients with revertant mosaicism in a single Wiskott-Aldrich syndrome family Blood, September 1, 2004; 104(5): 1270 - 1272. [Abstract] [Full Text] [PDF] A. Konno, T. Wada, S. H. Schurman, E. K. Garabedian, M. Kirby, S. M. Anderson, and F. Candotti Differential contribution of Wiskott-Aldrich syndrome protein to selective advantage in T- and B-cell lineages Blood, January 15, 2004; 103(2): 676 - 678. [Abstract] [Full Text] [PDF]

	Copyright © 2002 by American Society of Hematology         Online ISSN: 1528-0020