Pancytopenia in a Patient with Rendu-Osler-Weber Syndrome and Uncommon Vascular Abnormalities
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...
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Wiley
2016-01-01
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| Series: | Case Reports in Hematology |
| Online Access: | http://dx.doi.org/10.1155/2016/3016402 |
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| author | Nicolò Binello Antonio Gasbarrini Eleonora Gaetani |
| author_facet | Nicolò Binello Antonio Gasbarrini Eleonora Gaetani |
| author_sort | Nicolò Binello |
| collection | DOAJ |
| description | 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. |
| format | Article |
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| institution | Kabale University |
| issn | 2090-6560 2090-6579 |
| language | English |
| publishDate | 2016-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Hematology |
| spelling | doaj-art-9aa8dad8ce744426a8258ab50df1672f2025-02-03T01:12:33ZengWileyCase Reports in Hematology2090-65602090-65792016-01-01201610.1155/2016/30164023016402Pancytopenia in a Patient with Rendu-Osler-Weber Syndrome and Uncommon Vascular AbnormalitiesNicolò Binello0Antonio Gasbarrini1Eleonora Gaetani2Department of Internal Medicine, Agostino Gemelli University Hospital, Catholic University of Sacred Heart, Rome, ItalyDepartment of Internal Medicine, Agostino Gemelli University Hospital, Catholic University of Sacred Heart, Rome, ItalyDepartment of Internal Medicine, Agostino Gemelli University Hospital, Catholic University of Sacred Heart, Rome, ItalyRendu-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.http://dx.doi.org/10.1155/2016/3016402 |
| spellingShingle | Nicolò Binello Antonio Gasbarrini Eleonora Gaetani Pancytopenia in a Patient with Rendu-Osler-Weber Syndrome and Uncommon Vascular Abnormalities Case Reports in Hematology |
| title | Pancytopenia in a Patient with Rendu-Osler-Weber Syndrome and Uncommon Vascular Abnormalities |
| title_full | Pancytopenia in a Patient with Rendu-Osler-Weber Syndrome and Uncommon Vascular Abnormalities |
| title_fullStr | Pancytopenia in a Patient with Rendu-Osler-Weber Syndrome and Uncommon Vascular Abnormalities |
| title_full_unstemmed | Pancytopenia in a Patient with Rendu-Osler-Weber Syndrome and Uncommon Vascular Abnormalities |
| title_short | Pancytopenia in a Patient with Rendu-Osler-Weber Syndrome and Uncommon Vascular Abnormalities |
| title_sort | pancytopenia in a patient with rendu osler weber syndrome and uncommon vascular abnormalities |
| url | http://dx.doi.org/10.1155/2016/3016402 |
| work_keys_str_mv | AT nicolobinello pancytopeniainapatientwithrenduoslerwebersyndromeanduncommonvascularabnormalities AT antoniogasbarrini pancytopeniainapatientwithrenduoslerwebersyndromeanduncommonvascularabnormalities AT eleonoragaetani pancytopeniainapatientwithrenduoslerwebersyndromeanduncommonvascularabnormalities |