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Evolution of ADAMTS13 Antibodies in a Fatal Case of Thrombotic Thrombocytopenic Purpura

Overview
Journal Am J Hematol
Specialty Hematology
Date 2008 Jul 29
PMID 18661493
Citations 4
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Abstract

In a patient with fatal thrombotic thrombocytopenic purpura, the inhibitory activity of antibodies against ADAMTS13 rapidly escalated to extremely high levels despite daily plasma exchange and corticosteroid therapy. This increase was found to be because of a combination of higher antibody concentration and potency. Furthermore, during her course of the disease, the percentage of IgG(1) antibody progressively decreased whereas that of IgG(2) antibody increased, suggesting Th1-type cytokine response. These changes suggest that the course of TTP may be exacerbated by complex immune reactions. Further characterization of the factors contributing to this exacerbation may have important pathogenetic and therapeutic implications.

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References
1.
Estes D, Closser N, Allen G . IFN-gamma stimulates IgG2 production from bovine B cells costimulated with anti-mu and mitogen. Cell Immunol. 1994; 154(1):287-95. DOI: 10.1006/cimm.1994.1078. View

2.
Tsai H . Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion. Blood. 1996; 87(10):4235-44. View

3.
Tsai H, Lian E . Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med. 1998; 339(22):1585-94. PMC: 3159001. DOI: 10.1056/NEJM199811263392203. View

4.
Luken B, Kaijen P, Turenhout E, Kremer Hovinga J, van Mourik J, Fijnheer R . Multiple B-cell clones producing antibodies directed to the spacer and disintegrin/thrombospondin type-1 repeat 1 (TSP1) of ADAMTS13 in a patient with acquired thrombotic thrombocytopenic purpura. J Thromb Haemost. 2006; 4(11):2355-64. DOI: 10.1111/j.1538-7836.2006.02164.x. View

5.
Mayumi M, Kuritani T, Cooper M . T-cell regulation of IgG subclass expression by mitogen-induced plasma cells: soluble factors versus the T cells. J Clin Immunol. 1984; 4(4):287-94. DOI: 10.1007/BF00915296. View