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Evolution of ADAMTS13 antibodies in a fatal case of thrombotic thrombocytopenic purpura
Author(s) -
Dong Lingli,
Chandrasekaran Visalam,
Zhou Wenhua,
Tsai HanMou
Publication year - 2008
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.21217
Subject(s) - adamts13 , thrombotic thrombocytopenic purpura , exacerbation , medicine , antibody , immunology , immune system , platelet
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. Am. J. Hematol., 2008. © 2008 Wiley‐Liss, Inc.