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

Authors :
Serratrice C
Cox TM
Leguy-Seguin V
Morris E
Yousfi K
Monnet O
Sibert A
Allaham W
Belmatoug N
Source :
Journal of clinical medicine [J Clin Med] 2019 Feb 08; Vol. 8 (2). Date of Electronic Publication: 2019 Feb 08.
Publication Year :
2019

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.

Details

Language :
English
ISSN :
2077-0383
Volume :
8
Issue :
2
Database :
MEDLINE
Journal :
Journal of clinical medicine
Publication Type :
Academic Journal
Accession number :
30744043
Full Text :
https://doi.org/10.3390/jcm8020219