Defective ADAMTS13 synthesis as a possible consequence of NASH in an obese patient with recurrent thrombotic thrombocytopenic purpura.

Autor: Lombardi, Anna Maria, Fabris, Roberto, Berti de Marinis, Giulia, Marson, Piero, Navaglia, Filippo, Plebani, Mario, Vettor, Roberto, Fabris, Fabrizio
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Zdroj: European Journal of Haematology; Jun2014, Vol. 92 Issue 6, p497-501, 5p
Abstrakt: Objectives Thrombotic thrombocytopenic purpura ( TTP) is a rare and devastating hematologic disorder frequently associated with multiple organ failure and sometimes death. This syndrome is mainly associated with severe deficiency of ADAMTS13, a disintegrin and metalloprotease with thrombospondin ( TSP)-1 repeats, cleaving high molecular weight von Willebrand Factor ( ULVWF) multimers. Decreased plasma ADAMTS13 activity results in the accumulation of ULVWF multimers with consequent platelet activation. Recently, obesity has been considered as a potential independent risk factor for TTP, but the reason of this association is still unknown. Methods and results We describe an unusual case of fatal recurrent TTP in a morbid obese female with non-alcoholic steatohepatitis ( NASH) and severe ADAMTS13 activity deficiency due neither to an inhibitory autoantibody nor to a gene mutation. Conclusions Visceral obesity is associated with non-alcoholic fatty liver disease ( NAFLD) and NASH: we hypothesized that these conditions can influence ADAMTS13 antigen and activity. In fact, hepatic stellate cells ( HSC) are the main producers of ADAMTS13, and a decrease in ADAMTS13 activity has been reported in liver disease. [ABSTRACT FROM AUTHOR]
Databáze: Complementary Index
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