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A Rare Association of Congenital Asplenia with Jejunal Arteriovenous Malformation.
Am J Case Rep. 2017 Oct 19; 18:1118-1122.AJ

Abstract

BACKGROUND

Isolated congenital asplenia is a poorly understood and rare form of primary immunodeficiency, often associated with life-threatening infections. CASE REPORT We encountered a unique case of a 22-year-old asplenic male who presented with severe iron-deficiency anemia secondary to occult gastrointestinal bleeding since age 15. Our extensive work-up confirmed jejunal arteriovenous malformations as the source of the bleed. Six months after the treatment, the patient has reported no further episodes of gastrointestinal bleeding and his hemoglobin has remained stable.

CONCLUSIONS

A comprehensive literature review confirmed that this is the first reported case of adult congenital asplenia associated with arteriovenous malformation in the United States. The relationship of isolated congenital asplenia and arteriovenous malformation-associated bleeding remains unknown at this time; we postulate that this may be a congenital syndrome on its own. Obscure bleeding in the presence of rare anomalies such as asplenia should be investigated as one of the important causes of unexplained intestinal arteriovenous malformations.

Authors+Show Affiliations

Department of Internal Medicine, Henry Ford Macomb Hospital, Clinton Township, MI, USA.Department of Internal Medicine, Henry Ford Macomb Hospital, Clinton Township, MI, USA.Department of Medical Education, Henry Ford Macomb Hospital, Clinton Township, MI, USA.Department of Medical Education, Henry Ford Hospital, Detroit, MI, USA.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

29046517

Citation

Arnautovic, Jelena Z., et al. "A Rare Association of Congenital Asplenia With Jejunal Arteriovenous Malformation." The American Journal of Case Reports, vol. 18, 2017, pp. 1118-1122.
Arnautovic JZ, Mazhar A, Tereziu S, et al. A Rare Association of Congenital Asplenia with Jejunal Arteriovenous Malformation. Am J Case Rep. 2017;18:1118-1122.
Arnautovic, J. Z., Mazhar, A., Tereziu, S., & Gupta, K. (2017). A Rare Association of Congenital Asplenia with Jejunal Arteriovenous Malformation. The American Journal of Case Reports, 18, 1118-1122.
Arnautovic JZ, et al. A Rare Association of Congenital Asplenia With Jejunal Arteriovenous Malformation. Am J Case Rep. 2017 Oct 19;18:1118-1122. PubMed PMID: 29046517.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - A Rare Association of Congenital Asplenia with Jejunal Arteriovenous Malformation. AU - Arnautovic,Jelena Z, AU - Mazhar,Areej, AU - Tereziu,Stela, AU - Gupta,Kashvi, Y1 - 2017/10/19/ PY - 2017/10/20/entrez PY - 2017/10/20/pubmed PY - 2018/1/10/medline SP - 1118 EP - 1122 JF - The American journal of case reports JO - Am J Case Rep VL - 18 N2 - BACKGROUND Isolated congenital asplenia is a poorly understood and rare form of primary immunodeficiency, often associated with life-threatening infections. CASE REPORT We encountered a unique case of a 22-year-old asplenic male who presented with severe iron-deficiency anemia secondary to occult gastrointestinal bleeding since age 15. Our extensive work-up confirmed jejunal arteriovenous malformations as the source of the bleed. Six months after the treatment, the patient has reported no further episodes of gastrointestinal bleeding and his hemoglobin has remained stable. CONCLUSIONS A comprehensive literature review confirmed that this is the first reported case of adult congenital asplenia associated with arteriovenous malformation in the United States. The relationship of isolated congenital asplenia and arteriovenous malformation-associated bleeding remains unknown at this time; we postulate that this may be a congenital syndrome on its own. Obscure bleeding in the presence of rare anomalies such as asplenia should be investigated as one of the important causes of unexplained intestinal arteriovenous malformations. SN - 1941-5923 UR - https://www.unboundmedicine.com/medline/citation/29046517/A_Rare_Association_of_Congenital_Asplenia_with_Jejunal_Arteriovenous_Malformation. L2 - https://www.amjcaserep.com/download/index/idArt/903741 DB - PRIME DP - Unbound Medicine ER -