HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY 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
نویسندگان
چکیده
Silvia Ferrari,1 Friedrich Scheiflinger,1 Manfred Rieger,1 Geert Mudde,1 Martine Wolf,2,3 Paul Coppo,4 Jean-Pierre Girma,3 Elie Azoulay,5 Christian Brun-Buisson,6 Fadi Fakhouri,7 Jean-Paul Mira,8 Eric Oksenhendler,9 Pascale Poullin,10 Eric Rondeau,11 Nicolas Schleinitz,12 Benoit Schlemmer,5 Jean-Louis Teboul,13 Philippe Vanhille,14 Jean-Paul Vernant,15 Dominique Meyer,2,3 and Agnès Veyradier,2,3 for the French Clinical and Biological Network on Adult Thrombotic Microangiopathies
منابع مشابه
Potential for Recombinant ADAMTS13 as an Effective Therapy for Acquired Thrombotic Thrombocytopenic Purpura.
OBJECTIVE The metalloprotease ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) regulates the size of von Willebrand factor multimers. A deficiency in ADAMTS13 activity is associated with the life-threatening disease thrombotic thrombocytopenic purpura (TTP). The vast majority of patients have acquired TTP, where circulating anti-ADAMTS13 autoantibodie...
متن کاملHEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY Modulation of ADAMTS13 secretion and specific activity by a combination of common amino acid polymorphisms and a missense mutation
Sequence analysis of the ADAMTS13 locus of 2 patients with hereditary thrombotic thrombocytopenic purpura (TTP) revealed the homozygous presence of 4 single nucleotide polymorphisms (SNPs) (R7W, Q448E, P618A, A732V) and a rare missense mutation (R1336W). Analysis of the individual effect of any amino acid exchanges showed that several sequence variations can interact with each other, thereby al...
متن کاملReduced ADAMTS13 activity is associated with thrombotic risk in systemic lupus erythematosus.
BACKGROUND Severe deficiency of ADAMTS13 activity leads to von Willebrand factor (VWF) ultralarge multimers with high affinity for platelets, causing thrombotic thrombocytopenic purpura. Other pathological conditions with moderate ADAMTS13 activity exhibit a thrombotic risk. We examined the ADAMTS13 activity in systemic lupus erythematosus (SLE) and its value as a thrombotic biomarker. METHOD...
متن کاملPlatelet-delivered ADAMTS13 inhibits arterial thrombosis and prevents thrombotic thrombocytopenic purpura in murine models.
ADAMTS13 metalloprotease cleaves von Willebrand factor (VWF), thereby inhibiting platelet aggregation and arterial thrombosis. An inability to cleave ultralarge VWF resulting from hereditary or acquired deficiency of plasma ADAMTS13 activity leads to a potentially fatal syndrome, thrombotic thrombocytopenic purpura (TTP). Plasma exchange is the most effective initial therapy for TTP to date. He...
متن کاملAnticardiolipin Antibody in Patients with Behçet's Disease with and without Vascular Thrombosis
Objective: The clinical value of IgG anticardiolipin antibody in patients with Behçet's disease with or without vascular thrombosis was evaluated. Methods: IgG isotype of anticardiolipin (aCL) antibody was assessed in 40 Behçet's disease (BD) patients with venous or arterial thrombosis, 40 BD patients without venous or arterial thrombosis and 80 healthy subjects as controls. The levels of IgG...
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