Monatsschrift Kinderheilkunde

, Volume 152, Issue 4, pp 447–465

Immunthrombozytopenische Purpura bei Kindern

Diagnostik, Therapie und Kontroversen
Weiterbildung · Zertifizierte Fortbildung
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Zusammenfassung

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.

Schlüsselwörter

Immunthrombozytopenie Diagnose Therapie Kontroversen 

Immune thrombocytopenic purpura in children

Diagnosis, treatment and controversies

Abstract

Immune thrombocytopenic purpura is the commonest acquired haematological pathology. It is found in previously healthy children and generally has a benign course (remission in >80%). The acute form can be attributed to a previous virus infection with platelet-specific antibodies and resultant premature destruction of antibody-loaded thrombocytes in the reticuloendothelial system in about 60% of cases. In about 20% of affected children the thrombocytopenia persists for >6 months. The clinical course is variable and there are no uniformly present clinical characteristics, which makes it difficult to decide in individual cases whether the condition should be treated and, if so, what treatment should be selected. Treatments with corticosteroids, i.v. immunoglobulins, or i.v. anti-D-immunoglobulins are the most usual. Most doctors base their treatment of these children on the absolute thrombocyte count (<20×109 l−1), regardless of the clinical symptoms observed. They are guided in this by their fear of a possible life-threatening haemorrhage, although the risk of intracranial haemorrhage is only 0.1–0.9% and it has not yet been uniquivocally demonstrated that immediate induction therapy is beneficial when there are no relevant symptoms of bleeding.

Keywords

Immune thrombocytopenia Diagnosis Therapy Controversy 

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© Springer-Verlag 2004

Authors and Affiliations

  1. 1.Abteilung Hämatologie/OnkologieSt. Anna KinderspitalWienÖsterreich
  2. 2.Abteilung Hämatologie/OnkologieSt. Anna KinderspitalWienÖsterreich

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