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Blood, 15 January 2005, Vol. 105, No. 2, pp. 542-544. Prepublished online as a Blood First Edition Paper on September 14, 2004; DOI 10.1182/blood-2004-06-2096. This Article Full Text (PDF) All Versions of this Article: 2004-06-2096v1 105/2/542    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?  Previous Article  |  Next Article  Submitted June 2, 2004 Accepted August 26, 2004 Fatal congenital thrombotic thrombocytopenic purpura with apparent ADAMTS-13 inhibitor: in-vitro inhibition of ADAMTS-13 activity by hemoglobin Jan-Dirk Studt, Johanna A Kremer Hovinga, Gerhard Antoine, Martin Hermann, Manfred Rieger, Friedrich Scheiflinger, and Bernhard Lammle* Department of Hematology and Central Hematology Laboratory, Inselspital, University Hospital, Bern, Switzerland Baxter BioScience, Orth, Austria Department of Pediatrics, University Hospital, Tubingen, Germany * Corresponding author; email: bernhard.laemmle{at}insel.ch. Severe ADAMTS-13 deficiency in thrombotic thrombocytopenic purpura (TTP) either is constitutional and caused by ADAMTS13 mutations, or acquired and most often due to ADAMTS-13 inhibitory autoantibodies. In strongly hemolytic serum of a pediatric patient, diagnosed with TTP postmortem, ADAMTS-13 activity was less than 3%. Both parents had an ADAMTS-13 activity of ~50%. Sequencing of the ADAMTS13 gene revealed an intronic 687-2A>G substitution affecting exon 7, homozygous in the propositus and heterozygous in both parents, confirming constitutional ADAMTS-13 deficiency. ADAMTS-13 activity of normal plasma was inhibited by incubation with the propositus' serum, suggesting alloantibody formation to ADAMTS-13. However, immunoglobulin purified from serum had no ADAMTS-13 inhibitory effect whereas the immunoglobulin-depleted hemolytic serum inhibited ADAMTS-13 activity of normal plasma, suggesting an inhibitory effect of hemolysis products. Incubation of hemoglobin, recombinant and from lysed erythrocytes, with normal plasma revealed an ADAMTS-13 inhibitory effect at hemoglobin concentrations of 2mg/mL or higher. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Article in Blood Online: Hemoglobin versus ADAMTS13 David Motto Blood 2005 105: 436-437. [Full Text] [PDF] This article has been cited by other articles: Q. de Mast, E. Groot, P. B. Asih, D. Syafruddin, M. Oosting, S. Sebastian, B. Ferwerda, M. G. Netea, P. G. de Groot, A. J. A. M. van der Ven, et al. ADAMTS13 Deficiency with Elevated Levels of Ultra-Large and Active von Willebrand Factor in P. falciparum and P. vivax Malaria Am J Trop Med Hyg, March 1, 2009; 80(3): 492 - 498. [Abstract] [Full Text] [PDF] R. Palla, S. Lavoretano, R. Lombardi, I. Garagiola, M. Karimi, A. Afrasiabi, M. Ramzi, R. De Cristofaro, and F. Peyvandi The first deletion mutation in the TSP1-6 repeat domain of ADAMTS13 in a family with inherited thrombotic thrombocytopenic purpura Haematologica, February 1, 2009; 94(2): 289 - 293. [Abstract] [Full Text] [PDF] B.-J. H. van den Born, N. V. van der Hoeven, E. Groot, P. J. Lenting, J. C.M. Meijers, M. Levi, and G. A. van Montfrans Association Between Thrombotic Microangiopathy and Reduced ADAMTS13 Activity in Malignant Hypertension Hypertension, April 1, 2008; 51(4): 862 - 866. [Abstract] [Full Text] [PDF] S. Ferrari, F. Scheiflinger, M. Rieger, G. Mudde, M. Wolf, P. Coppo, J.-P. Girma, E. Azoulay, C. Brun-Buisson, F. Fakhouri, et al. Prognostic value of anti-ADAMTS13 antibody features (Ig isotype, titer, and inhibitory effect) in a cohort of 35 adult French patients undergoing a first episode of thrombotic microangiopathy with undetectable ADAMTS13 activity Blood, April 1, 2007; 109(7): 2815 - 2822. [Abstract] [Full Text] [PDF] R. F. Machado, A. Anthi, M. H. Steinberg, D. Bonds, V. Sachdev, G. J. Kato, A. M. Taveira-DaSilva, S. K. Ballas, W. Blackwelder, X. Xu, et al. N-terminal pro-brain natriuretic peptide levels and risk of death in sickle cell disease. JAMA, July 19, 2006; 296(3): 310 - 318. [Abstract] [Full Text] [PDF] M. Rieger, P. M. Mannucci, J. A. K. Hovinga, A. Herzog, G. Gerstenbauer, C. Konetschny, K. Zimmermann, I. Scharrer, F. Peyvandi, M. Galbusera, et al. ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases Blood, August 15, 2005; 106(4): 1262 - 1267. [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]

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