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This Article Full Text Full Text (PDF) 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 Nishimura, J.-i. Articles by Kinoshita, T. Search for Related Content PubMed PubMed Citation Articles by Nishimura, J.-i. Articles by Kinoshita, T. Related Collections Hematopoiesis and Stem Cells Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  Blood, 15 April 2002, Vol. 99, No. 8, pp. 2748-2751 HEMATOPOIESIS Long-term support of hematopoiesis by a single stem cell clone in patients with paroxysmal nocturnal hemoglobinuria Jun-ichi Nishimura, Toshiyuki Hirota, Yuzuru Kanakura, Takashi Machii, Takashi Kageyama, Shoichi Doi, Hiroshi Wada, Toru Masaoka, Yoshio Kanayama, Hiroshi Fujii, Nobumasa Inoue, Maki Kuwayama, Norimitsu Inoue, Kazuhito Ohishi, and Taroh Kinoshita From the Department of Immunoregulation/Research Institute for Microbial Diseases and the Research Foundation for Microbial Diseases, Osaka University; the Department of Hematology and Oncology, Osaka University Medical School; Ikoma General Hospital, Nara; Osaka Red Cross Hospital; Second Department of Internal Medicine, Hyogo College of Medicine; Osaka Medical Center for Cancer; Asiya Municipal Hospital, Hyogo; Southern Wakayama National Hospital; and Osaka National Hospital, Japan. Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hematopoietic stem cell disorder characterized by clonal blood cells that are deficient in glycosylphosphatidylinositol-anchored proteins because of somatic mutations of the PIG-A gene. Many patients with PNH have more than one PNH clone, but it is unclear whether a single PNH clone remains dominant or minor clones eventually become dominant. Furthermore, it is unknown how many hematopoietic stem cells (HSCs) sustain hematopoiesis and how long a single HSC can support hematopoiesis in humans. To understand dynamics of HSCs, we reanalyzed the PIG-A gene mutations in 9 patients 6 to 10 years after the previous analyses. The proportion of affected peripheral blood polymorphonuclear cells (PMNs) in each patient was highly variable; it increased in 2 (from 50% and 65% to 98% and 97%, respectively), was stable in 4 (changed less than 20%), and diminished in 3 (94%, 99%, and 98% to 33%, 57%, and 43%, respectively) patients. The complexity of these results reflects the high variability of the clinical course of PNH. In all patients, the previously predominant clone was still present and dominant. Therefore, one stem cell clone can sustain hematopoiesis for 6 to 10 years in patients with PNH. Two patients whose affected PMNs decreased because of a decline of the predominant PNH clone and who have been followed up for 24 and 31 years now have an aplastic condition, suggesting that aplasia is a terminal feature of PNH. © 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: R. Hu, G. L. Mukhina, S. Piantadosi, J. P. Barber, R. J. Jones, and R. A. Brodsky PIG-A mutations in normal hematopoiesis Blood, May 15, 2005; 105(10): 3848 - 3854. [Abstract] [Full Text] [PDF]

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