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Recombinant ADAMTS13 normalizes von Willebrand factor-cleaving activity in plasma of acquired TTP patients by overriding inhibitory antibodies.
Plaimauer, B; Kremer Hovinga, J A; Juno, C; Wolfsegger, M J; Skalicky, S; Schmidt, M; Grillberger, L; Hasslacher, M; Knöbl, P; Ehrlich, H; Scheiflinger, F.
Afiliación
  • Plaimauer B; Baxter Innovations GmbH, Wagramer Strasse, Vienna, Austria.
J Thromb Haemost ; 9(5): 936-44, 2011 May.
Article en En | MEDLINE | ID: mdl-21294825
ABSTRACT

BACKGROUND:

Severe deficiency of the von Willebrand factor (VWF)-cleaving protease ADAMTS13 as observed in acquired thrombotic thrombocytopenic purpura (TTP) is caused by inhibitory and non-inhibitory autoantibodies directed against the protease. Current treatment with plasma exchange is considered to remove circulating antibodies and to concurrently replenish the deficient enzyme.

OBJECTIVES:

To explore the use of recombinant ADAMTS13 (rADAMTS13) as a potential therapeutic agent in acquired TTP, we investigated its efficacy in normalizing VWF-cleaving activity in the presence of ADAMTS13 inhibitors.

METHODS:

Thirty-six plasma samples from TTP patients were adjusted to predefined inhibitor titers, and recovery of ADAMTS13 activity was analyzed following supplementation with rADAMTS13.

RESULTS:

We showed a linear relation between the inhibitor titer measured and effective rADAMTS13 concentration necessary for reconstitution of VWF-cleaving activity in the presence of neutralizing autoantibodies.

CONCLUSIONS:

Our results support the further investigation of the potential therapeutic applicability of rADAMTS13 as an adjunctive therapy in acquired TTP.
Asunto(s)

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Púrpura Trombocitopénica Trombótica / Factor de von Willebrand / Proteínas ADAM / Anticuerpos Tipo de estudio: Prognostic_studies Límite: Adult / Aged / Female / Humans / Male / Middle aged Idioma: En Revista: J Thromb Haemost Asunto de la revista: HEMATOLOGIA Año: 2011 Tipo del documento: Article País de afiliación: Austria

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Púrpura Trombocitopénica Trombótica / Factor de von Willebrand / Proteínas ADAM / Anticuerpos Tipo de estudio: Prognostic_studies Límite: Adult / Aged / Female / Humans / Male / Middle aged Idioma: En Revista: J Thromb Haemost Asunto de la revista: HEMATOLOGIA Año: 2011 Tipo del documento: Article País de afiliación: Austria