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Blood, 15 November 2003, Vol. 102, No. 10, pp. 3587-3591. Prepublished online as a Blood First Edition Paper on July 31, 2003; DOI 10.1182/blood-2003-01-0009. This Article Full Text Full Text (PDF) All Versions of this Article: 2003-01-0009v1 102/10/3587    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 Hall, C. Articles by Hillmen, P. Search for Related Content PubMed PubMed Citation Articles by Hall, C. Articles by Hillmen, P. Related Collections Hematopoiesis and Stem Cells Hemostasis, Thrombosis, and Vascular Biology Red Cells Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY Primary prophylaxis with warfarin prevents thrombosis in paroxysmal nocturnal hemoglobinuria (PNH) Claire Hall, Stephen Richards, and Peter Hillmen From the Haematological Malignancy Diagnostic Service (HMDS), Leeds General Infirmary, Leeds, United Kingdom. Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia in which venous thrombosis is the most common cause of death. Here we address the risk factors for thrombosis and the role of warfarin prophylaxis in PNH. The median follow-up of 163 PNH patients was 6 years (range, 0.2-38 years). Of the patients, 29 suffered thromboses, with a 10-year incidence of 23%. There were 9 patients who presented with thrombosis, and in the remainder the median time to thrombosis was 4.75 years (range, 3 months-15 years). The 10-year risk of thrombosis in patients with large PNH clones (PNH granulocytes > 50%) was 44% compared with 5.8% with small clones (P < .01). Patients with large PNH clones and no contraindication to anticoagulation were offered warfarin. There were no thromboses in the 39 patients who received primary prophylaxis. In comparison, 56 patients with large clones and not taking warfarin had a 10-year thrombosis rate of 36.5% (P = .01). There were 2 serious hemorrhages in more than 100 patient-years of warfarin therapy. Large PNH granulocyte clones are predictive of venous thrombosis, although the exact cut-off for clone size is still to be determined. Primary prophylaxis with warfarin in PNH prevents thrombosis with acceptable risks. (Blood. 2003;102:3587-3591) CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: A Gupta, R Al Fulaij, R. Gupta, G Gupta, and R Marouf Development of paroxysmal nocturnal haemoglobinuria in systemic lupus erythematosus: an unusual cause of portal vein thrombosis Lupus, July 1, 2009; 18(8): 743 - 746. [Abstract] [PDF] R. A. Brodsky How I treat paroxysmal nocturnal hemoglobinuria Blood, June 25, 2009; 113(26): 6522 - 6527. [Abstract] [Full Text] [PDF] F. E. Craig and K. A. Foon Flow cytometric immunophenotyping for hematologic neoplasms Blood, April 15, 2008; 111(8): 3941 - 3967. [Abstract] [Full Text] [PDF] P. Hillmen, P. Muus, U. Duhrsen, A. M. Risitano, J. Schubert, L. Luzzatto, H. Schrezenmeier, J. Szer, R. A. Brodsky, A. Hill, et al. Effect of the complement inhibitor eculizumab on thromboembolism in patients with paroxysmal nocturnal hemoglobinuria Blood, December 1, 2007; 110(12): 4123 - 4128. [Abstract] [Full Text] [PDF] M. D. Cappellini Coagulation in the Pathophysiology of Hemolytic Anemias Hematology, January 1, 2007; 2007(1): 74 - 78. [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] A. Hill, P. Hillmen, S. J. Richards, D. Elebute, J. C. Marsh, J. Chan, C. F. Mojcik, and R. P. Rother Sustained response and long-term safety of eculizumab in paroxysmal nocturnal hemoglobinuria Blood, October 1, 2005; 106(7): 2559 - 2565. [Abstract] [Full Text] [PDF] R. P. Rother, L. Bell, P. Hillmen, and M. T. Gladwin The Clinical Sequelae of Intravascular Hemolysis and Extracellular Plasma Hemoglobin: A Novel Mechanism of Human Disease JAMA, April 6, 2005; 293(13): 1653 - 1662. [Abstract] [Full Text] [PDF] W. F. Rosse, P. Hillmen, and A. D. Schreiber Immune-Mediated Hemolytic Anemia Hematology, January 1, 2004; 2004(1): 48 - 62. [Abstract] [Full Text] [PDF] G. C. Bagby, J. M. Lipton, E. M. Sloand, and C. A. Schiffer Marrow Failure Hematology, January 1, 2004; 2004(1): 318 - 336. [Abstract] [Full Text] [PDF]

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