Recurrent Severe Hypochromic Microcytic Anemia Multiple Blood Transfusions and Skin Lesions: Blue Rubber Bleb Nevus Syndrome
Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare congenital disease of unknown etiology, presents with multiple venous malformations in skin and visceral organs particularly Gastrointestinal Tract (GIT). Skin lesions are asymptomatic, GIT bleb can bleeds. A 10-year female patient was presented with...
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Krishna Vishwa Vidyapeeth (Deemed to be University), Karad
2018-10-01
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| Series: | Journal of Krishna Institute of Medical Sciences University |
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| Online Access: | http://www.jkimsu.com/jkimsu-vol7no4/JKIMSU,%20Vol.%207,%20No.%204,%20October-December%202018%20Page%2096-100.pdf |
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| author | Anirban Chatterjee Subham Bhattacharya |
| author_facet | Anirban Chatterjee Subham Bhattacharya |
| author_sort | Anirban Chatterjee |
| collection | DOAJ |
| description | Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare congenital disease of unknown etiology, presents with multiple venous malformations in skin and visceral organs particularly Gastrointestinal Tract (GIT). Skin lesions are asymptomatic, GIT bleb can bleeds. A 10-year female patient was presented with recurrent severe anemia that necessities several times hospital admission and blood transfusion. She was investigated for cause of anemia in every admission and investigations were repetition of same tests of previous admissions including invasive diagnostic procedure like bone marrow aspiration. Investigations have confirmed Iron Deficiency Anemia (IDA). She had small bluish-black compressible blebs in trunk and limbs since early age;
the numbers of blebs are increasing with age. Endoscopy revealed small blebs in GIT. After blood transfusion and management of IDA, she has sent to gastrointestinal surgery department for specialized endoscopic management of GIT blebs. In this case the cause of IDA has defined chronic bleeding GIT blebs of BRBNS. We are reporting this rare disease to emphasize
that skin blebs appeared from early childhood are typical of BRBNS that are indicator of vascular malformation of GIT as chronic bleeder in BRBNS. This understanding can contribute for right diagnosis at earlier point so several hospitalization, multiple
transfusions, the cost of investigations could have been avoided. In addition, supportive management may be initiated early. |
| format | Article |
| id | doaj-art-655204a417cb4eee944e3fc5eb095491 |
| institution | OA Journals |
| issn | 2231-4261 2231-4261 |
| language | English |
| publishDate | 2018-10-01 |
| publisher | Krishna Vishwa Vidyapeeth (Deemed to be University), Karad |
| record_format | Article |
| series | Journal of Krishna Institute of Medical Sciences University |
| spelling | doaj-art-655204a417cb4eee944e3fc5eb0954912025-08-20T01:55:00ZengKrishna Vishwa Vidyapeeth (Deemed to be University), KaradJournal of Krishna Institute of Medical Sciences University2231-42612231-42612018-10-01070496100Recurrent Severe Hypochromic Microcytic Anemia Multiple Blood Transfusions and Skin Lesions: Blue Rubber Bleb Nevus SyndromeAnirban Chatterjee 0Subham Bhattacharya1Department of Pediatric Medicine, Institute of Post Graduate Medical Education and Research, Kolkata- 700020 (West Bengal) IndiaDepartment of Pediatric Medicine, Institute of Post Graduate Medical Education and Research, Kolkata- 700020 (West Bengal) IndiaBlue Rubber Bleb Nevus Syndrome (BRBNS) is a rare congenital disease of unknown etiology, presents with multiple venous malformations in skin and visceral organs particularly Gastrointestinal Tract (GIT). Skin lesions are asymptomatic, GIT bleb can bleeds. A 10-year female patient was presented with recurrent severe anemia that necessities several times hospital admission and blood transfusion. She was investigated for cause of anemia in every admission and investigations were repetition of same tests of previous admissions including invasive diagnostic procedure like bone marrow aspiration. Investigations have confirmed Iron Deficiency Anemia (IDA). She had small bluish-black compressible blebs in trunk and limbs since early age; the numbers of blebs are increasing with age. Endoscopy revealed small blebs in GIT. After blood transfusion and management of IDA, she has sent to gastrointestinal surgery department for specialized endoscopic management of GIT blebs. In this case the cause of IDA has defined chronic bleeding GIT blebs of BRBNS. We are reporting this rare disease to emphasize that skin blebs appeared from early childhood are typical of BRBNS that are indicator of vascular malformation of GIT as chronic bleeder in BRBNS. This understanding can contribute for right diagnosis at earlier point so several hospitalization, multiple transfusions, the cost of investigations could have been avoided. In addition, supportive management may be initiated early.http://www.jkimsu.com/jkimsu-vol7no4/JKIMSU,%20Vol.%207,%20No.%204,%20October-December%202018%20Page%2096-100.pdfIron Deficiency AnemiaSkin NeviBlue Rubber Bleb Nevus Syndrome |
| spellingShingle | Anirban Chatterjee Subham Bhattacharya Recurrent Severe Hypochromic Microcytic Anemia Multiple Blood Transfusions and Skin Lesions: Blue Rubber Bleb Nevus Syndrome Journal of Krishna Institute of Medical Sciences University Iron Deficiency Anemia Skin Nevi Blue Rubber Bleb Nevus Syndrome |
| title | Recurrent Severe Hypochromic Microcytic Anemia Multiple Blood Transfusions and Skin Lesions: Blue Rubber Bleb Nevus Syndrome |
| title_full | Recurrent Severe Hypochromic Microcytic Anemia Multiple Blood Transfusions and Skin Lesions: Blue Rubber Bleb Nevus Syndrome |
| title_fullStr | Recurrent Severe Hypochromic Microcytic Anemia Multiple Blood Transfusions and Skin Lesions: Blue Rubber Bleb Nevus Syndrome |
| title_full_unstemmed | Recurrent Severe Hypochromic Microcytic Anemia Multiple Blood Transfusions and Skin Lesions: Blue Rubber Bleb Nevus Syndrome |
| title_short | Recurrent Severe Hypochromic Microcytic Anemia Multiple Blood Transfusions and Skin Lesions: Blue Rubber Bleb Nevus Syndrome |
| title_sort | recurrent severe hypochromic microcytic anemia multiple blood transfusions and skin lesions blue rubber bleb nevus syndrome |
| topic | Iron Deficiency Anemia Skin Nevi Blue Rubber Bleb Nevus Syndrome |
| url | http://www.jkimsu.com/jkimsu-vol7no4/JKIMSU,%20Vol.%207,%20No.%204,%20October-December%202018%20Page%2096-100.pdf |
| work_keys_str_mv | AT anirbanchatterjee recurrentseverehypochromicmicrocyticanemiamultiplebloodtransfusionsandskinlesionsbluerubberblebnevussyndrome AT subhambhattacharya recurrentseverehypochromicmicrocyticanemiamultiplebloodtransfusionsandskinlesionsbluerubberblebnevussyndrome |