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Semin Thromb Hemost 2022; 48(05): 529-541
DOI: 10.1055/s-0041-1741571
Review Article

Gastrointestinal Bleeding in Congenital Bleeding Disorders

Amir Samii
1   Department of Hematology and Blood Transfusion, School of Allied Medicine, Iran University of Medical Sciences, Tehran, Iran
,
Mahshaad Norouzi
2   Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine, Tehran, Iran
,
Abbas Ahmadi
3   Cellular and Molecular Research Center, Research Institute for Health Development, Kurdistan University of Medical Sciences, Sanandaj, Iran
,
Akbar Dorgalaleh
1   Department of Hematology and Blood Transfusion, School of Allied Medicine, Iran University of Medical Sciences, Tehran, Iran
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Abstract

Gastrointestinal bleeding (GIB) is serious, intractable, and potentially life-threatening condition. There is considerable heterogeneity in GIB phenotypes among congenital bleeding disorders (CBDs), making GIB difficult to manage. Although GIB is rarely encountered in CBDs, its severity in some patients makes the need for a comprehensive and precise assessment of underlying factors and management approaches imperative. Initial evaluation of GIB begins with assessment of hematological status; GIB should be ruled out in patients with chronic anemia, and in presentations that include hematemesis, hematochezia, or melena. High-risk patients with recurrent GIB require urgent interventions such as replacement therapy for treatment of coagulation factor deficiency (CFD). However, the best management strategy for CFD-related bleeding remains controversial. While several investigations have identified CBDs as potential risk factors for GIB, research has focused on assessing the risks for individual factor deficiencies and other CBDs. This review highlights recent findings on the prevalence, management strategies, and alternative therapies of GIB related to CFDs, and platelet disorders.

Keywords

gastrointestinal bleeding - congenital bleeding disorders - hemorrhage - replacement therapy - coagulation factors deficiency


Publication History

Article published online:
12 January 2022

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