Immunthrombozytopenische Purpura bei Kindern

Springer Science and Business Media LLC - Tập 152 - Trang 447-465 - 2004
H. Gadner1, S. Shukry-Schulz1, A. Zoubek1
1Abteilung Hämatologie/Onkologie, St. Anna Kinderspital, Wien, Österreich

Tóm tắt

Die immunthrombozytopenische Purpura ist die häufigste erworbene Blutungskrankheit. Sie tritt bei zuvor gesunden Kindern auf und verläuft in der Regel benigne (Remission >80%). Die akute Form kann in etwa 60% auf eine vorausgegangene Virusinfektion mit plättchenspezifischen Antikörpern und damit vorzeitiger Zerstörung antikörperbeladener Thrombozyten im RES zurückgeführt werden. Bei etwa 20% der Kinder persistiert die Thrombozytopenie >6 Monate. Die Variabilität des klinischen Verlaufs und das Fehlen einheitlicher klinischer Merkmale machen die Entscheidung, ob und wie behandelt werden soll, im Einzelfall nicht einfach. Üblicherweise wird mit Kortikosteroiden, i. v. Immunglobulinen oder i. v. Anti-D-Immunglobulinen behandelt. Die meisten Ärzte therapieren Kinder vorwiegend auf Basis von absoluten Thrombozytenzahlen (<20×109/l) unabhängig von vorhandenen klinischen Symptomen. Dabei lassen sie sich von der Sorge um die Möglichkeit einer lebensbedrohlichen Blutung leiten, obwohl das Risiko einer intrakraniellen Blutung nur bei 0,1–0,9% liegt und bislang nicht erwiesen ist, dass eine sofortige Initialtherapie bei fehlender relevanter Blutungssymptomatik von Vorteil ist.

Tài liệu tham khảo

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