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Blood, 1 June 2004, Vol. 103, No. 11, pp. 4195-4197. Prepublished online as a Blood First Edition Paper on March 4, 2004; DOI 10.1182/blood-2003-11-3888. This Article Full Text Full Text (PDF) All Versions of this Article: 2003-11-3888v1 2003-11-3888v2 103/11/4195    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 Studt, J.-D. Articles by Lämmle, B. Search for Related Content PubMed PubMed Citation Articles by Studt, J.-D. Articles by Lämmle, B. Related Collections Hemostasis, Thrombosis, and Vascular Biology Immunobiology Brief Reports Clinical Trials and Observations Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY Brief report Familial acquired thrombotic thrombocytopenic purpura: ADAMTS13 inhibitory autoantibodies in identical twins Jan-Dirk Studt, Johanna A. Kremer Hovinga, Radovan Radonic, Vladimir Gasparovic, Dragutin Ivanovic, Marijan Merkler, Urs Wirthmueller, Clemens Dahinden, Miha Furlan, and Bernhard Lämmle From the Central Hematology Laboratory, Inselspital, University Hospital, Bern, Switzerland; the Department of Internal Medicine, University Hospital, Zagreb, Croatia; and the Institute of Immunology, Inselspital, University Hospital, Bern, Switzerland. Thrombotic thrombocytopenic purpura (TTP) either occurs in a congenital form caused by ADAMTS13 gene mutations or it is acquired and most often due to ADAMTS13 inhibitory autoantibodies. In congenital TTP siblings are often affected, while acquired TTP occurs sporadically and familial clustering has not been described so far. We report identical twin sisters suffering from acquired TTP due to immunoglobulin G (IgG) autoantibodies inactivating ADAMTS13, suggesting an important role of hitherto unidentified genetic determinants of ADAMTS13 inhibitor formation. These cases also demonstrate that familial clustering is not sufficient for unambiguously diagnosing hereditary ADAMTS13 deficiency and congenital TTP. (Blood. 2004;103:4195-4197) CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Article in Blood Online: ADAMTS13 and TTP: the clot thickens James N. George and Sara K. Vesely Blood 2004 103: 3997-3998. [Full Text] [PDF] This article has been cited by other articles: B. Lammle, J. A. Kremer Hovinga, and J. N. George Acquired thrombotic thrombocytopenic purpura: ADAMTS13 activity, anti-ADAMTS13 autoantibodies and risk of recurrent disease Haematologica, February 1, 2008; 93(2): 172 - 177. [Full Text] [PDF] G. G. Levy, D. G. Motto, and D. Ginsburg ADAMTS13 turns 3 Blood, July 1, 2005; 106(1): 11 - 17. [Abstract] [Full Text] [PDF] M. Noris, S. Bucchioni, M. Galbusera, R. Donadelli, E. Bresin, F. Castelletti, J. Caprioli, S. Brioschi, F. Scheiflinger, G. Remuzzi, et al. Complement Factor H Mutation in Familial Thrombotic Thrombocytopenic Purpura with ADAMTS13 Deficiency and Renal Involvement J. Am. Soc. Nephrol., May 1, 2005; 16(5): 1177 - 1183. [Abstract] [Full Text] [PDF] J. E. Sadler, J. L. Moake, T. Miyata, and J. N. George Recent Advances in Thrombotic Thrombocytopenic Purpura Hematology, January 1, 2004; 2004(1): 407 - 423. [Abstract] [Full Text] [PDF]

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