SEARCH:   Advanced

Prepublished online as a Blood First Edition Paper on August 8, 2002; DOI 10.1182/blood-2002-03-0799. This Article Full Text (PDF) All Versions of this Article: 2002-03-0799v1 100/12/3897    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 Wang, H. Articles by Nakao, S. Search for Related Content PubMed PubMed Citation Articles by Wang, H. Articles by Nakao, S. Social Bookmarking                   What's this? Submitted March 18, 2002 Accepted July 15, 2002 Clinical significance of a minor population of paroxysmal nocturnal hemoglobinuria-type cells in bone marrow failure syndrome Hongbo Wang, Tatsuya Chuhjo, Shizuka Yasue, Mitsuhiro Omine, and Shinji Nakao* * Corresponding author; email: snakao{at}med3.m.kanazawa-u.ac.jp. A minor population of blood cells deficient of glycosylphosphatidylinositol (GPI)-anchored membrane proteins is often detected in patients with aplastic anemia (AA) although the clinical significance of such paroxysmal nocturnal hemoglobinuria (PNH)-type cells remains unclear. To clarify this issue, we studied a total of 164 patients with myelodysplastic syndrome (MDS) for the presence of CD55-CD59- granulocytes and red blood cells using sensitive flow cytometry. Among the different subgroups of MDS, a significant increase (i.e., at least 0.003%) of PNH-type cells was detected only in 21 of 119 patients with refractory anemia (RA) and this frequency (17.6%) of RA patients with increased PNH-type cells (PNH+ patients) was much lower than that which we previously reported for AA patients (52.0%). PNH+ RA patients had distinct clinical features compared to RA patients without increased PNH-type cells (PNH- patients), such as less-pronounced morphologic abnormalities of blood cells, more severe thrombocytopenia, lower rates of karyotypic abnormalities (4.8% vs 32.8%) and of progression to acute leukemias (0% vs 6.2%), a higher probability of responding to cyclosporine therapy (77.8% vs 0%), and a higher incidence of possessing the HLA-DR15 (90.5% vs 18.5%). These data indicate that the presence of a minor population of PNH-type cells suggests a benign type of bone marrow failure, probably due to an immunologic mechanism. Peripheral blood should be tested using sensitive flow cytometry for the presence of PNH-type cells in all patients with bone marrow failure before treatment in order to choose an appropriate therapy. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: R. A. Brodsky How I treat paroxysmal nocturnal hemoglobinuria Blood, June 25, 2009; 113(26): 6522 - 6527. [Abstract] [Full Text] [PDF] N. S. Young Paroxysmal nocturnal hemoglobinuria and myelodysplastic sydromes: clonal expansion of PIG-A-mutant hematopoietic cells in bone marrow failure Haematologica, January 1, 2009; 94(1): 3 - 7. [Full Text] [PDF] 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] R. A. Brodsky Narrative Review: Paroxysmal Nocturnal Hemoglobinuria: The Physiology of Complement-Related Hemolytic Anemia Ann Intern Med, April 15, 2008; 148(8): 587 - 595. [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] C. Sugimori, T. Chuhjo, X. Feng, H. Yamazaki, A. Takami, M. Teramura, H. Mizoguchi, M. Omine, and S. Nakao Minor population of CD55-CD59- blood cells predicts response to immunosuppressive therapy and prognosis in patients with aplastic anemia Blood, February 15, 2006; 107(4): 1308 - 1314. [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] J. W. Vardiman Hematopathological Concepts and Controversies in the Diagnosis and Classification of Myelodysplastic Syndromes Hematology, January 1, 2006; 2006(1): 199 - 204. [Abstract] [Full Text] [PDF] D. P. Steensma and J. M. Bennett The Myelodysplastic Syndromes: Diagnosis and Treatment Mayo Clin. Proc., January 1, 2006; 81(1): 104 - 130. [Abstract] [Full Text] [PDF] C. Parker, M. Omine, S. Richards, J.-i. Nishimura, M. Bessler, R. Ware, P. Hillmen, L. Luzzatto, N. Young, T. Kinoshita, et al. Diagnosis and management of paroxysmal nocturnal hemoglobinuria Blood, December 1, 2005; 106(12): 3699 - 3709. [Full Text] [PDF] J. P. Maciejewski and A. M. Risitano Aplastic Anemia: Management of Adult Patients Hematology, January 1, 2005; 2005(1): 110 - 117. [Abstract] [Full Text] [PDF] C. Sugimori, T. Chuhjo, X. Lu, X. Feng, K. Ishiyama, H. Yamazaki, A. Takami, and S. Nakao A Minor Population of CD55-CD59- Blood Cells Detected by Two-Color Flow Cytometry in Aplastic Anemia Patients: A Reliable Marker for Good Response to Immunosuppressive Therapy. Blood (ASH Annual Meeting Abstracts), November 16, 2004; 104(11): 1593 - 1593. [Abstract] [Full Text] H. Yamazaki, T. Chuhjo, C. Sugimori, K. Ishiyama, and S. Nakao Identification of a Subset of Aplastic Anemia Patients Highly Responsive to Danazol. Blood (ASH Annual Meeting Abstracts), November 16, 2004; 104(11): 2817 - 2817. [Abstract] X. Feng, T. Chuhjo, C. Sugimori, T. Kotani, X. Lu, A. Takami, H. Takamatsu, H. Yamazaki, and S. Nakao Diazepam-binding inhibitor-related protein 1: a candidate autoantigen in acquired aplastic anemia patients harboring a minor population of paroxysmal nocturnal hemoglobinuria-type cells Blood, October 15, 2004; 104(8): 2425 - 2431. [Abstract] [Full Text] [PDF] D. P. Steensma, V. Viprakasit, A. Hendrick, D. K. Goff, J. Leach, R. J. Gibbons, and D. R. Higgs Deletion of the {alpha}-globin gene cluster as a cause of acquired {alpha}-thalassemia in myelodysplastic syndrome Blood, February 15, 2004; 103(4): 1518 - 1520. [Abstract] [Full Text] [PDF] K. Ishiyama, T. Chuhjo, H. Wang, A. Yachie, M. Omine, and S. Nakao Polyclonal hematopoiesis maintained in patients with bone marrow failure harboring a minor population of paroxysmal nocturnal hemoglobinuria-type cells Blood, August 15, 2003; 102(4): 1211 - 1216. [Abstract] [Full Text] [PDF] G. Mufti, A. F. List, S. D. Gore, and A. Y.L. Ho Myelodysplastic Syndrome Hematology, January 1, 2003; 2003(1): 176 - 199. [Abstract] [Full Text] [PDF]

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