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Blood, 15 February 2006, Vol. 107, No. 4, pp. 1308-1314. Prepublished online as a Blood First Edition Paper on September 22, 2005; DOI 10.1182/blood-2005-06-2485. This Article Full Text Full Text (PDF) All Versions of this Article: 2005-06-2485v1 107/4/1308    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 Sugimori, C. Articles by Nakao, S. Search for Related Content PubMed PubMed Citation Articles by Sugimori, C. Articles by Nakao, S. Related Collections Hematopoiesis and Stem Cells Red Cells Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  CLINICAL TRIALS AND OBSERVATIONS Minor population of CD55-CD59- blood cells predicts response to immunosuppressive therapy and prognosis in patients with aplastic anemia Chiharu Sugimori, Tatsuya Chuhjo, Xingmin Feng, Hirohito Yamazaki, Akiyoshi Takami, Masanao Teramura, Hideaki Mizoguchi, Mitsuhiro Omine, and Shinji Nakao From the Cellular Transplantation Biology, Division of Cancer Medicine, Kanazawa University Graduate School of Medical Science, Ishikawa; the Preventive Environment Unit, Kanazawa University Hospital, Ishikawa; the Division of Hematology, Tokyo Women's Medical University, Tokyo; and the Division of Hematology, Fujigaoka Hospital, Showa University School of Medicine, Yokohama, Japan. We investigated the clinical significance of a minor population of paroxysmal nocturnal hemoglobinuria (PNH)-type blood cells in patients with acquired aplastic anemia (AA). We quantified CD55-CD59- granulocytes and red blood cells (RBCs) in peripheral blood from 122 patients with recently diagnosed AA and correlated numbers of PNH-type cells and responses to immunosuppressive therapy (IST). Flow cytometry detected 0.005% to 23.1% of GPI-AP- cells in 68% of patients with AA. Sixty-eight of 83 (91%) patients with an increased proportion of PNH-type cells (PNH+) responded to antithymocyte globulin (ATG) + cyclosporin (CsA) therapy, whereas 18 of 39 (48%) without such an increase (PNH-) responded. Failure-free survival rates were significantly higher (64%) among patients with PNH+ than patients with PNH- (12%) at 5 years, although overall survival rates were comparable between the groups. Numbers of PNH-type and normal-type cells increased in parallel among most patients with PNH+ who responded to IST, suggesting that these cells are equally sensitive to immune attack. These results indicate that a minor population of PNH-type cells represents a reliable marker of a positive IST response and a favorable prognosis among patients with AA. Furthermore, immune attack against hematopoietic stem cells that allows PNH clonal expansion might occur only at the onset of AA. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: S. A. Wang, O. Pozdnyakova, J. L. Jorgensen, L. J. Medeiros, D. Stachurski, M. Anderson, A. Raza, and B. A. Woda Detection of paroxysmal nocturnal hemoglobinuria clones in patients with myelodysplastic syndromes and related bone marrow diseases, with emphasis on diagnostic pitfalls and caveats Haematologica, January 1, 2009; 94(1): 29 - 37. [Abstract] [Full Text] [PDF] H. Y. Lim, M. Farley, C. Wittwer, and C. Parker Characteristics of Laboratory Screening Suggest That Paroxysmal Nocturnal Hemoglobinuria (PNH) Is Perceived Primarily as a Bone Marrow Failure Syndrome Rather Than as a Hemolytic Anemia Blood (ASH Annual Meeting Abstracts), November 16, 2008; 112(11): 4107 - 4107. [Abstract] K. Mochizuki, C. Sugimori, Z. Qi, X. Lu, A. Takami, K. Ishiyama, Y. Kondo, H. Yamazaki, H. Okumura, and S. Nakao Expansion of donor-derived hematopoietic stem cells with PIGA mutation associated with late graft failure after allogeneic stem cell transplantation Blood, September 1, 2008; 112(5): 2160 - 2162. [Abstract] [Full Text] [PDF] R. A. Brodsky Paroxysmal Nocturnal Hemoglobinuria: Stem Cells and Clonality Hematology, January 1, 2008; 2008(1): 111 - 115. [Abstract] [Full Text] [PDF] H. Takamatsu, X. Feng, T. Chuhjo, X. Lu, C. Sugimori, K. Okawa, M. Yamamoto, S. Iseki, and S. Nakao Specific antibodies to moesin, a membrane-cytoskeleton linker protein, are frequently detected in patients with acquired aplastic anemia Blood, March 15, 2007; 109(6): 2514 - 2520. [Abstract] [Full Text] [PDF] A. Bacigalupo Aplastic Anemia: Pathogenesis and Treatment Hematology, January 1, 2007; 2007(1): 23 - 28. [Abstract] [Full Text] [PDF] N. Inoue, T. Izui-Sarumaru, Y. Murakami, Y. Endo, J.-I. Nishimura, K. Kurokawa, M. Kuwayama, H. Shime, T. Machii, Y. Kanakura, et al. Molecular basis of clonal expansion of hematopoiesis in 2 patients with paroxysmal nocturnal hemoglobinuria (PNH) Blood, December 15, 2006; 108(13): 4232 - 4236. [Abstract] [Full Text] [PDF] N. S. Young, R. T. Calado, and P. Scheinberg Current concepts in the pathophysiology and treatment of aplastic anemia Blood, October 15, 2006; 108(8): 2509 - 2519. [Abstract] [Full Text] [PDF] R. A. Brodsky New Insights into Paroxysmal Nocturnal Hemoglobinuria Hematology, January 1, 2006; 2006(1): 24 - 28. [Abstract] [Full Text] [PDF] N. S. Young Pathophysiologic Mechanisms in Acquired Aplastic Anemia Hematology, January 1, 2006; 2006(1): 72 - 77. [Abstract] [Full Text] [PDF]

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