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
