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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| Main Authors: | , |
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| Format: | Article |
| Language: | English |
| Published: |
Krishna Vishwa Vidyapeeth (Deemed to be University), Karad
2018-10-01
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| Series: | Journal of Krishna Institute of Medical Sciences University |
| Subjects: | |
| Online Access: | http://www.jkimsu.com/jkimsu-vol7no4/JKIMSU,%20Vol.%207,%20No.%204,%20October-December%202018%20Page%2096-100.pdf |
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| Summary: | 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. |
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| ISSN: | 2231-4261 2231-4261 |