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Spontaneous thrombosis of hepatic aneurysms in an infant with Wiskott-Aldrich syndrome

Gungor, Tayfun
Ozsahin, Hulya
Published in Pediatric Hematology & Oncology. 2009, vol. 26, no. 4, p. 261-266
Abstract Vasculitides and aneurysm formation are well-known complications in Wiskott-Aldrich syndrome (WAS), most often appearing later in life, usually in second decade. The authors report the case of a 5-month-old boy with a genetically and phenotypically severe Wiskott-Aldrich syndrome and sequential formation and spontaneous thrombosis of hepatic aneurysms. This case demonstrates that aneurysm formation may develop early in the course of severe WAS phenotypes. Because of the progressive nature of these manifestations, surgical or interventional procedures are not advisable. Early allogeneic hematopoietic stem cell transplantation (HSCT) should be considered before the manifestation of irreversible organ damage.
Keywords Aneurysm/etiology/surgery/*ultrasonographyFatal OutcomeHematopoietic Stem Cell TransplantationHepatic Artery/*ultrasonographyHumansInfantMaleThrombosis/etiology/surgery/*ultrasonographyTomography, X-Ray ComputedUltrasonography, DopplerWiskott-Aldrich Syndrome/*complications/surgery
PMID: 19437328
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Research groups Radiologie neuro-diagnostique et neuro-interventionnelle en pédiatrie (842)
Plateforme d'oncologie et d'hématologie pédiatrique (907)
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MERLINI, Laura et al. Spontaneous thrombosis of hepatic aneurysms in an infant with Wiskott-Aldrich syndrome. In: Pediatric Hematology & Oncology, 2009, vol. 26, n° 4, p. 261-266. https://archive-ouverte.unige.ch/unige:19964

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Deposited on : 2012-04-23

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