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


Merlini, L; Hanquinet, S; Gungor, T; Ozsahin, H (2009). Spontaneous thrombosis of hepatic aneurysms in an infant with Wiskott-Aldrich syndrome. Pediatric Hematology-Oncology, 26(4):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.

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.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Children's Hospital Zurich > Medical Clinic
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:2009
Deposited On:17 Feb 2010 15:06
Last Modified:05 Apr 2016 13:54
Publisher:Informa Healthcare
ISSN:0888-0018
Additional Information:Informa Healthcare access full text
Publisher DOI:https://doi.org/10.1080/08880010902900239
PubMed ID:19437328

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