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Blood, 1 June 2004, Vol. 103, No. 11, pp. 4195-4197. Prepublished online as a Blood First Edition Paper on February 24, 2004March 4, 2004; DOI 10.1182/blood-2003-11-3888. This Article 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 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 Lammle, B. Search for Related Content PubMed PubMed Citation Articles by Studt, J.-D. Articles by Lammle, B. Related Collections Related Article in Blood Online Social Bookmarking                   What's this? Submitted November 13, 2003 Accepted February 12, 2004 Familial acquired thrombotic thrombocytopenic purpura: ADAMTS-13 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 Lammle* Central Hematology Laboratory, Inselspital, University Hospital, Bern, Switzerland Department of Internal Medicine, University Hospital, Zagreb, Croatia (Hrvatska) Institute of Immunology, Inselspital, University Hospital, Bern, Switzerland * Corresponding author; email: bernhard.laemmle{at}insel.ch. Thrombotic thrombocytopenic purpura (TTP) occurs either in a congenital form caused by ADAMTS13 gene mutations, or is acquired and most often due to ADAMTS-13 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 IgG autoantibodies inactivating ADAMTS-13, suggesting an important role of hitherto unidentified genetic determinants of ADAMTS-13 inhibitor formation. These cases also demonstrate that familial clustering is not sufficient for unambiguously diagnosing hereditary ADAMTS-13 deficiency and congenital TTP. 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] S. C. Meyer, C. R. Largiader, S. Jin, X. L. Zheng, C. A. Dahinden, J. N. George, B. Lammle, and J. A. Kremer Hovinga The ADAMTS13 Gene as the Immunological Culprit in Acute Acquired TTP - First Evidence of Genetic Out-Breeding Depression in Humans. Blood (ASH Annual Meeting Abstracts), November 16, 2007; 110(11): 277 - 277. [Abstract] 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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