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Idiopathische thrombozytopenische Purpura im Kindesalter

Idiopathic thrombocytopenic purpura in childhood

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Zusammenfassung

Die idiopathische thrombozytopenische Purpura (ITP) ist eine Blutungskrankheit, die durch eine verkürzte Lebensdauer der Thrombozyten charakterisiert ist. Sie ist heterogen ausgeprägt und wird durch endogene und erworbene Faktoren beeinflusst. Sie ist eine Ausschlussdiagnose, deren Differenzialdiagnose stets bedacht werden muss. Die Unkenntnis der Ätiologie und der Mangel an klinischen Daten aus kontrollierten prospektiven Studien haben Kontroversen hinsichtlich Diagnose und Behandlung zur Folge. Die bisherigen prospektiven Therapiestudien haben die Beschleunigung des Thrombozytenanstiegs zum Ziel. Diese Zielsetzung wird oft in den klinischen Alltag übertragen, ohne dass bisher gezeigt werden konnte, dass ein rascher Thrombozytenanstieg von klinischem Wert ist. Bei der Behandlung des Patienten mit ITP ist meist eine Vorbeugung vor fatalen Blutungen beabsichtigt. Diese sind aber im Kindesalter sehr selten. Die Therapieziele im klinischen Alltag, aber auch in klinischen Studien müssen überdacht werden. Andere wichtige Gesichtspunkte sind Blutungen, die Lebensqualität des Patienten und seiner Angehörigen, Nebenwirkungen von Medikamenten und ökonomische Aspekte.

Abstract

Idiopathic thrombocytopenic purpura (ITP) is a bleeding disorder characterised by shortened platelet survival. ITP is heterogeneous and is affected by endogenous and acquired factors. The diagnosis is one of exclusion, meaning that differential diagnosis is always important. Because of the unknown aetiology and the lack of clinical data collected from controlled prospective studies, the diagnosis and management of patients with ITP are controversial. The objective in prospective trials has been to alter the velocity of platelet increase. This study goal is frequently extrapolated to clinical situations, but no evidence has been found to show that a fast increase in the platelet count is clinically significant. The treatment of a patient with ITP is generally directed at preventing life-threatening haemorrhages, which are very rare in childhood. The goals of management and the study end-points in clinical trials should be re-evaluated. Important aspects are bleeding, quality of life for the patient and adherents, side effects of drugs and economic matters.

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  1. Abteilung Onkologie/Hämatologie, Universitätskinderspital beider Basel, Römergasse 8, 4005, Basel, Schweiz

    T. Kühne & P. Imbach

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  1. T. Kühne
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  2. P. Imbach
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Correspondence to T. Kühne.

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Kühne, T., Imbach, P. Idiopathische thrombozytopenische Purpura im Kindesalter. Monatsschr Kinderheilkd 154, 533–539 (2006). https://doi.org/10.1007/s00112-006-1345-0

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