SEARCH:   Advanced

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 Eldor, A. Articles by Rachmilewitz, E. A. Search for Related Content PubMed PubMed Citation Articles by Eldor, A. Articles by Rachmilewitz, E. A. Related Collections Hemostasis, Thrombosis, and Vascular Biology Red Cells Review Articles Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  Blood, 1 January 2002, Vol. 99, No. 1, pp. 36-43 REVIEW ARTICLE The hypercoagulable state in thalassemia Amiram Eldor and Eliezer A. Rachmilewitz From the Institute of Hematology, Tel-Aviv Sourasky Medical Center, Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel; and Department of Hematology, Edith Wolfson Medical Center, Holon, Israel. Thalassemia is a congenital hemolytic disorder caused by a partial or complete deficiency of - or -globin chain synthesis. Homozygous carriers of -globin gene defects suffer from severe anemia and other serious complications from early childhood. The disease is treated by chronic blood transfusion. However, this can cause severe iron overload resulting in progressive organ failure. Some forms of  thalassemia are also associated with a similar clinical picture. Despite the difficulties associated with treatment, standards of care for thalassemic patients have improved in recent years, resulting in almost doubling of the average life expectancy. As a consequence, additional previously undescribed, complications are now being recognized. In particular, profound hemostatic changes have been observed in patients with -thalassemia major (-TM) and -thalassemia intermedia (-TI) and also in patients with  thalassemia (hemoglobin H disease). The presence of a higher than normal incidence of thromboembolic events, mainly in -TI, and the existence of prothrombotic hemostatic anomalies in the majority of the patients, even from a very young age, have led to the recognition of the existence of a chronic hypercoagulable state in thalassemic patients. Despite the appearance of numerous publications on the frequent occurrence of thromboembolic complications in thalassemia, this complication has not been emphasized or comprehensively reviewed. This review summarizes the current literature and discusses possible mechanisms of the lifelong hypercoagulable state that exists in thalassemia. © 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. Setiabudy, P. A. Wahidiyat, and L. Setiawan Platelet Aggregation and Activation in Thalassemia Major Patients in Indonesia Clinical and Applied Thrombosis/Hemostasis, July 1, 2008; 14(3): 346 - 351. [Abstract] [PDF] A. Habib, C. Kunzelmann, W. Shamseddeen, F. Zobairi, J.-M. Freyssinet, and A. Taher Elevated levels of circulating procoagulant microparticles in patients with {beta}-thalassemia intermedia Haematologica, June 1, 2008; 93(6): 941 - 942. [Full Text] [PDF] E. J. van Beers, B. L. F. van Eck-Smit, M. R. Mac Gillavry, C. F. J. van Tuijn, J. W. J. van Esser, D. P. M. Brandjes, M. C. Kappers-Klunne, A. J. Duits, B. J. Biemond, J.-J. B. Schnog, et al. Large and Medium-Sized Pulmonary Artery Obstruction Does Not Play a Role of Primary Importance in the Etiology of Sickle-Cell Disease-Associated Pulmonary Hypertension Chest, March 1, 2008; 133(3): 646 - 652. [Abstract] [Full Text] [PDF] M. T. Gladwin and G. J. Kato Hemolysis-associated hypercoagulability in sickle cell disease: the plot (and blood) thickens! Haematologica, January 1, 2008; 93(1): 1 - 3. [Full Text] [PDF] A. Aessopos, M. Kati, and D. Farmakis Heart disease in thalassemia intermedia: a review of the underlying pathophysiology Haematologica, May 1, 2007; 92(5): 658 - 665. [Abstract] [Full Text] [PDF] E. Stoyanova, M. Trudel, H. Felfly, D. Garcia, and G. Cloutier Characterization of circulatory disorders in {beta}-thalassemic mice by noninvasive ultrasound biomicroscopy Physiol Genomics, March 14, 2007; 29(1): 84 - 90. [Abstract] [Full Text] [PDF] V. Brankovic-Sreckovic, V. Milic Rasic, V. Djordjevic, M. Kuzmanovic, and S. Pavlovic Arterial Ischemic Stroke in a Child with {beta}-Thalassemia Trait and Methylentetrahydrofolate Reductase Mutation J Child Neurol, February 1, 2007; 22(2): 208 - 210. [Abstract] [PDF] M. D. Cappellini Coagulation in the Pathophysiology of Hemolytic Anemias Hematology, January 1, 2007; 2007(1): 74 - 78. [Abstract] [Full Text] [PDF] E. Vichinsky Hemoglobin E Syndromes Hematology, January 1, 2007; 2007(1): 79 - 83. [Abstract] [Full Text] [PDF] J. Valles, M. Pinon, M. A. Dasi, A. Moscardo, M. P. Fuset, A. Latorre, and M.-T. Santos Contribution of Platelets and Erythrocytes to Thrombin Generation in Patients with {beta}-Thalassemia. Blood (ASH Annual Meeting Abstracts), November 16, 2006; 108(11): 3820 - 3820. [Abstract] [PDF] A Phrommintikul, A Sukonthasarn, R Kanjanavanit, and W Nawarawong Splenectomy: a strong risk factor for pulmonary hypertension in patients with thalassaemia Heart, October 1, 2006; 92(10): 1467 - 1472. [Abstract] [Full Text] [PDF] E. C. Attar and R. P. Hasserjian Case 14-2006 -- a 25-year-old woman with anemia and iron overload. N. Engl. J. Med., May 11, 2006; 354(19): 2047 - 2056. [Full Text] [PDF] D. Farmakis, A. Polonifi, S. Deftereos, M. Tsironi, I. Papaioannou, and A. Aessopos Aortic Valve Replacement in a Patient With Thalassemia Intermedia Ann. Thorac. Surg., February 1, 2006; 81(2): 737 - 739. [Abstract] [Full Text] [PDF] M. Sanefuji, S. Ohga, R. Kira, and T. Yoshiura Moyamoya Syndrome in a Splenectomized Patient With {beta}-Thalassemia Intermedia J Child Neurol, January 1, 2006; 21(1): 75 - 77. [Abstract] [PDF] M. J. Weiss Handling heme Blood, October 1, 2005; 106(7): 2225 - 2226. [Full Text] [PDF] D. Rund and E. Rachmilewitz {beta}-Thalassemia N. Engl. J. Med., September 15, 2005; 353(11): 1135 - 1146. [Full Text] [PDF] P. G. Firth Anaesthesia for peculiar cells--a century of sickle cell disease Br. J. Anaesth., September 1, 2005; 95(3): 287 - 299. [Abstract] [Full Text] [PDF] A. Aessopos, D. Farmakis, S. Deftereos, M. Tsironi, S. Tassiopoulos, I. Moyssakis, and M. Karagiorga Thalassemia Heart Disease: A Comparative Evaluation of Thalassemia Major and Thalassemia Intermedia Chest, May 1, 2005; 127(5): 1523 - 1530. [Abstract] [Full Text] [PDF] D. N. Lewis, A. Nyska, K. Johnson, D. E. Malarkey, S. Ward, M. Streicker, S. Shabat, S. Peddada, and M. Nyska 2-Butoxyethanol Female-Rat Model of Hemolysis and Disseminated Thrombosis: X-Ray Characterization of Osteonecrosis and Growth-Plate Suppression Toxicol Pathol, February 1, 2005; 33(2): 272 - 282. [Abstract] [Full Text] [PDF] V. Atichartakarn, S. Chuncharunee, P. Chandanamattha, K. Likittanasombat, and K. Aryurachai Correction of hypercoagulability and amelioration of pulmonary arterial hypertension by chronic blood transfusion in an asplenic hemoglobin E/{beta}-thalassemia patient Blood, April 1, 2004; 103(7): 2844 - 2846. [Abstract] [Full Text] [PDF] A. R. Cohen, R. Galanello, D. J. Pennell, M. J. Cunningham, and E. Vichinsky Thalassemia Hematology, January 1, 2004; 2004(1): 14 - 34. [Abstract] [Full Text] [PDF] D. H. K. Chui, S. Fucharoen, and V. Chan Hemoglobin H disease: not necessarily a benign disorder Blood, February 1, 2003; 101(3): 791 - 800. [Full Text] [PDF]

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