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Blood, 15 January 2005, Vol. 105, No. 2, pp. 443-452. Prepublished online as a Blood First Edition Paper on September 9, 2004; DOI 10.1182/blood-2004-07-2792. This Article Full Text Full Text (PDF) All Versions of this Article: 2004-07-2792v1 105/2/443    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 Steensma, D. P. Articles by Higgs, D. R. Search for Related Content PubMed PubMed Citation Articles by Steensma, D. P. Articles by Higgs, D. R. Related Collections Neoplasia Red Cells Free Research Articles Review Articles Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  REVIEW ARTICLES Acquired -thalassemia in association with myelodysplastic syndrome and other hematologic malignancies David P. Steensma, Richard J. Gibbons, and Douglas R. Higgs From the Medical Research Council (MRC) Molecular Haematology Unit, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, United Kingdom; and the Division of Hematology, Mayo Clinic, Rochester, MN. Abnormalities of hemoglobin synthesis are usually inherited but may also arise as a secondary manifestation of another disease, most commonly hematologic neoplasia. Acquired hemoglobin disorders can be seen in any population and are not restricted to areas of the world with high incidences of inherited hemoglobinopathies. In fact, the acquired hemoglobinopathies may be more readily recognized where inherited hemoglobin abnormalities are rare and less likely to cause diagnostic confusion. Acquired -thalassemia is the best characterized of the acquired red blood cell disorders in patients with hematologic malignancy, and it is almost always associated with a myelodysplastic syndrome (MDS). At least 2 molecular mechanisms for acquired -thalassemia are now recognized: acquired deletion of the -globin gene cluster limited to the neoplastic clone and, more commonly, inactivating somatic mutations of the trans-acting chromatin-associated factor ATRX, which cause dramatic down-regulation of -globin gene expression. Here we review the clinical, hematologic, and molecular genetic features of -thalassemia arising in a clonal myeloid disorder, and we discuss howATRX might affect gene expression in normal and abnormal hematopoiesis through epigenetic mechanisms. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: M. Czader and A. Orazi Therapy-Related Myeloid Neoplasms Am J Clin Pathol, September 1, 2009; 132(3): 410 - 425. [Abstract] [Full Text] [PDF] H. P. J. Voon and J. Vadolas Controlling {alpha}-globin: a review of {alpha}-globin expression and its impact on {beta}-thalassemia Haematologica, December 1, 2008; 93(12): 1868 - 1876. [Abstract] [Full Text] [PDF] A. N. Schechter Hemoglobin research and the origins of molecular medicine Blood, November 15, 2008; 112(10): 3927 - 3938. [Abstract] [Full Text] [PDF] A. Zebisch and H. Sill Severe Hemolysis As Presenting Sign of Acute Erythroleukemia J. Clin. Oncol., January 10, 2008; 26(2): 330 - 331. [Full Text] [PDF] D. P. Steensma and A. Tefferi Anemia in the Elderly: How Should We Define It, When Does It Matter, and What Can Be Done? Mayo Clin. Proc., August 1, 2007; 82(8): 958 - 966. [Abstract] [Full Text] [PDF] L. Guo, Y. Ma, R. Ward, V. Castranova, X. Shi, and Y. Qian Constructing Molecular Classifiers for the Accurate Prognosis of Lung Adenocarcinoma. Clin. Cancer Res., June 1, 2006; 12(11): 3344 - 3354. [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] D. P. Steensma, G. W. Dewald, T. L. Lasho, H. L. Powell, R. F. McClure, R. L. Levine, D. G. Gilliland, and A. Tefferi The JAK2 V617F activating tyrosine kinase mutation is an infrequent event in both "atypical" myeloproliferative disorders and myelodysplastic syndromes Blood, August 15, 2005; 106(4): 1207 - 1209. [Abstract] [Full Text] [PDF] D. P. Steensma and A. F. List Genetic Testing in the Myelodysplastic Syndromes: Molecular Insights Into Hematologic Diversity Mayo Clin. Proc., May 1, 2005; 80(5): 681 - 698. [Abstract] [PDF] R. J. Gibbons Histone modifying and chromatin remodelling enzymes in cancer and dysplastic syndromes Hum. Mol. Genet., April 15, 2005; 14(suppl_1): R85 - R92. [Abstract] [Full Text] [PDF]

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