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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 (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 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 Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted July 22, 2004 Accepted August 21, 2004 Acquired Thalassemia in Association with Myelodysplastic Syndrome and Other Hematologic Malignancies David P Steensma, Richard J Gibbons, and Douglas R Higgs* MRC Molecular Haematology Unit, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Headington, Oxford, United Kingdom; Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA MRC Molecular Haematology Unit, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Headington, Oxford, United Kingdom * Corresponding author; email: doug.higgs{at}imm.ox.ac.uk. 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 a high incidence 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 most well-characterized of the acquired red cell disorders described in the setting of hematologic malignancy, and is almost always associated with a myelodysplastic syndrome (MDS). At least two 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 that cause dramatic down-regulation of globin gene expression. Here we review the clinical, hematologic, and molecular genetic features of thalassemia arising in the setting of a clonal myeloid disorder, and we discuss how ATRX might affect gene expression in normal and abnormal hematopoiesis via 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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