Splenic Artery Aneurysms, A Rare Complication of Type 1 Gaucher Disease: Report of Five Cases.

Abstract

Type 1 Gaucher disease is a rare genetic lysosomal disorder due to acid betaglucosidase deficiency. The main features are thrombocytopenia, anemia, hepatosplenomegaly and complex skeletal disease. Complications include pulmonary hypertension, cirrhosis and splenic infarction; comorbidities, such as autoimmune phenomena, B-cell malignancies and Parkinson disease also occur. Visceral aneurysms have been only rarely noted in Gaucher disease. We report the retrospective data from patients with Gaucher disease type 1 and splenic arterial aneurysm. We describe the different outcomes of a giant splenic arterial aneurysm in five patients with type 1 Gaucher disease and discuss the main possible pathophysiological explanations. Aneurysms of the splenic artery are rare in Gaucher disease but are probably greatly under-reported.

• Authors: Christine Serratrice, Timothy M Cox, Vanessa Leguy-Seguin, Elizabeth Morris, Karima Yousfi, Olivier Monnet, Annie Sibert, Wassim Allaham, Nadia Belmatoug
• Journal: Journal of clinical medicine (J Clin Med) Vol. 8 Issue 2 (Feb 08 2019) ISSN: 2077-0383 [Print] Switzerland
• PMID: 30744043 (Publication Type: Case Reports)