Zusammenfassung
Das sinusoidale Obstruktionssyndrom (SOS), auch als „veno-occlusive disease“ (VOD) bezeichnet, ist eine seltene, potenziell letal verlaufende Durchblutungsstörung der Lebersinusoide, der ursächlich in den meisten Fällen eine toxische Schädigung im Rahmen einer Stammzelltransplantation vorangeht. Die Diagnose ist oft schwierig und basiert auf klinischen Kriterien sowie auf Laborparametern und Bildgebung. Klinische Parameter sind unter anderem Ikterus, Gewichtszunahme und eine schmerzhafte Hepatomegalie. Bildgebend ist v. a. die abdominelle Sonographie von Bedeutung. Therapeutisch werden milde Verläufe supportiv behandelt, bei schwer erkrankten Patienten hat ein frühzeitiger Therapiebeginn mit Defibrotid positive Effekte gezeigt.
Abstract
Hepatic sinusoidal obstruction syndrome (SOS), also known as veno-occlusive disease (VOD), is a rare, potentially life-threatening microcirculatory disorder of the hepatic sinuses that in most cases occurs after a prior hematopoietic stem cell transplantation. Diagnosis is often difficult and based on clinical criteria, laboratory parameters and imaging. Clinical parameters are, among others, jaundice, weight gain and painful hepatomegaly. Abdominal ultrasound serves as an important imaging tool. Mild courses receive symptomatic treatment, while for severe cases the early administration of defibrotide has shown positive effects.
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M. Schulz und A. Wree erklären, dass sie sich bei der Erstellung des Beitrags nicht von wirtschaftlichen Interessen leiten ließen. Es werden die folgenden potenziellen Interessenkonflikte offengelegt: Forschungsarbeiten im Labor von Prof. Wree wurde von Intercept gefördert, er hat Vortrags- oder Beratungshonorare von Falk, Intercept, Gilead und Daiichi-Sankyo erhalten.
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Schulz, M., Wree, A. Sinusoidales Obstruktionssyndrom und venookklusive Lebererkrankung – Diagnose und Therapiemöglichkeiten. Gastroenterologe 16, 102–108 (2021). https://doi.org/10.1007/s11377-021-00505-7
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DOI: https://doi.org/10.1007/s11377-021-00505-7