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Pancytopenia in a Patient with Rendu-Osler-Weber Syndrome and Uncommon Vascular Abnormalities
Joint Authors
Gaetani, Eleonora
Binello, Nicolò
Gasbarrini, Antonio
Source
Issue
Vol. 2016, Issue 2016 (31 Dec. 2016), pp.1-2, 2 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2016-10-10
Country of Publication
Egypt
No. of Pages
2
Main Subjects
Abstract EN
Rendu-Osler-Weber syndrome, or hereditary hemorrhagic teleangiectasia (HHT), is a rare autosomal dominant vascular disorder, characterized by multiple mucocutaneous teleangiectases with recurrent nasal and gastrointestinal bleedings and/or solid-organ arteriovenous shunts.
We describe the first case to our knowledge of pancytopenia in a 53-year-old patient, with a known history of HHT and recurrent nasal and gastrointestinal bleedings, who was found to have a major splenic artery aneurysm and other uncommon vascular abnormalities.
In the absence of other evident causes of pancytopenia, hypersplenism was diagnosed.
The patient underwent coil embolization of the splenic artery aneurysm, followed by rapid and sustained increase of white blood cell and platelet count.
Splenic artery aneurysms are extremely uncommon in HHT as only anecdotal cases have been reported to date.
However, we believe that the aneurysm critically contributed to the progression of splenomegaly and the development of pancytopenia.
American Psychological Association (APA)
Binello, Nicolò& Gasbarrini, Antonio& Gaetani, Eleonora. 2016. Pancytopenia in a Patient with Rendu-Osler-Weber Syndrome and Uncommon Vascular Abnormalities. Case Reports in Hematology،Vol. 2016, no. 2016, pp.1-2.
https://search.emarefa.net/detail/BIM-1100843
Modern Language Association (MLA)
Binello, Nicolò…[et al.]. Pancytopenia in a Patient with Rendu-Osler-Weber Syndrome and Uncommon Vascular Abnormalities. Case Reports in Hematology No. 2016 (2016), pp.1-2.
https://search.emarefa.net/detail/BIM-1100843
American Medical Association (AMA)
Binello, Nicolò& Gasbarrini, Antonio& Gaetani, Eleonora. Pancytopenia in a Patient with Rendu-Osler-Weber Syndrome and Uncommon Vascular Abnormalities. Case Reports in Hematology. 2016. Vol. 2016, no. 2016, pp.1-2.
https://search.emarefa.net/detail/BIM-1100843
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1100843