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001-es BibID:BIBFORM035991
Első szerző:Réti Marienn
Cím:Complement activation in thrombotic thrombocytopenic purpura / Réti M., Farkas P., Csuka D., Rázsó K., Schlammadinger Á., Udvardy M. L., Madách K., Domján G., Bereczki C., Reusz G. S., Szabó A. J., Prohászka Z.
Dátum:2012
ISSN:1538-7933
Megjegyzések:Ultra-large von Willebrand factor and deficiency of its cleaving protease are important factors in the events leading to thrombotic microangiopathy; however, the mechanisms involved are only partly understood. Whereas pathological activation of the alternative complement pathway is linked to atypical hemolytic uremic syndrome, the role of complement activation in thrombotic thrombocytopenic purpura (TTP) is unknown. The aim of this study was to investigate whether signs of complement activation are characteristic of TTP. PATIENTS AND METHODS: Twenty-three patients with TTP (18 women, median age 38 years) and 17 healthy controls (13 women, median age 38 years) were included. Complement parameters (C3, Factors H, I, B and total alternative pathway activity) together with complement activation fragments (C3a) or complexes (C1rs-INH, C3bBbP, sC5b9) were measured by ELISA or RID. ADAMTS13 activity and anti-ADAMTS13 inhibitory antibodies were measured by the VWF-FRET73 assay. RESULTS: Increased levels of C3a, and SC5b9 were observed in TTP during acute episodes, as compared with healthy controls. Decreased complement C3 levels indicative of complement consumption occurred in 15% of acute TTP patients. Significant decrease of complement activation products C3a and SC5b9 was observed during plasma exchange (PEX). The sustained presence of anti-ADAMTS13 inhibitory antibodies in complete remission was associated with increased complement activation.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
ADAMTS13 inhibitor
complement activation
TTP
Megjelenés:Journal Of Thrombosis And Haemostasis. - 10 : 5 (2012), p. 791-798. -
További szerzők:Farkas P. Csuka D. Molnárné Rázsó Katalin (1966-) (belgyógyász, haematológus, klinikai onkológus) Schlammadinger Ágota (1971-) (belgyógyász, haematológus) Udvardy Miklós László (1977-) (orvos, tudományos segédmunkatárs) Madách Krisztina Domján Gyula Bereczki Csaba Reusz György Szabó A. J. Prohászka Zoltán
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