eISSN: 2084-9869
ISSN: 1233-9687
Polish Journal of Pathology
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SCImago Journal & Country Rank
2/2021
vol. 72
 
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abstract:
Case report

Bean’s syndrome (blue rubber bleb nevus syndrome – BRBNS) as a gastrointestinal bleeding – case report and review of the literature

Małgorzata Lenarcik
1
,
Jacek Pachlewski
1
,
Andrzej Mróz
2

1.
Department of Gastroenterology, Hepatology and Clinical Oncology, Centre of Medical Postgraduate Education, Warsaw, Poland
2.
Department of Pathomorphology, Centre of Medical Postgraduate Education, Warsaw, Poland
Pol J Pathol 2021; 72 (2): 190-194
Online publish date: 2021/09/30
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BRBNS is a rare syndrome of vascular malformations caused by the TEK mutation associated with numerous lesions of the skin and gastrointestinal tract. We present a case report of 41 year old man with severe anemia with recurrent bleedings. The detailed clinical, endoscopical and histopathological description is given as a wide range of differential diagnosis of vascular lesions based on pathophysiology and updated classification of vascular lesions. Clinicopathological diagnosis and treatment options of BRBNS are discussed.
keywords:

Bean’s syndrome (BRBNS), vascular malformation, TEK mutation, GLUT1, hemangioma

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