TY - JOUR
T1 - Advantages and limits of ADAMTS13 testing in the prognostic assessment of thrombotic thrombocytopenic purpura
AU - Mannucci, Pier Mannuccio
AU - Franchini, Massimo
PY - 2012/3
Y1 - 2012/3
N2 - Thrombotic thrombocytopenic purpura (TTP) is a rare but severe disease characterized by mechanical hemolytic anemia and consumptive thrombocytopenia leading to disseminated microvascular thrombosis that causes signs and symptoms of organ ischemia and functional damage. After the elucidation of the pathophysiology of TTP, thanks to the demonstration of the congenital or acquired (autoimmune) plasma deficiency of the von Willebrand factor cleaving metalloprotease A Disintegrin And Metalloprotease with Thrombo. Spondin 1 repeats (ADAMTS13), a number of laboratory assays for measuring ADAMTS13 and related autoantibodies have been developed. Current knowledge on the diagnostic and prognostic value of ADAMTS13 and anti-ADAMTS13 assays is summarized in this review.
AB - Thrombotic thrombocytopenic purpura (TTP) is a rare but severe disease characterized by mechanical hemolytic anemia and consumptive thrombocytopenia leading to disseminated microvascular thrombosis that causes signs and symptoms of organ ischemia and functional damage. After the elucidation of the pathophysiology of TTP, thanks to the demonstration of the congenital or acquired (autoimmune) plasma deficiency of the von Willebrand factor cleaving metalloprotease A Disintegrin And Metalloprotease with Thrombo. Spondin 1 repeats (ADAMTS13), a number of laboratory assays for measuring ADAMTS13 and related autoantibodies have been developed. Current knowledge on the diagnostic and prognostic value of ADAMTS13 and anti-ADAMTS13 assays is summarized in this review.
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U2 - 10.1016/j.lpm.2011.10.025
DO - 10.1016/j.lpm.2011.10.025
M3 - Article
C2 - 22244722
AN - SCOPUS:84857440816
VL - 41
JO - Nouvelle Presse Medicale
JF - Nouvelle Presse Medicale
SN - 0755-4982
IS - 3 PART 2
ER -