Gastrointestinal Bleeding in Congenital Bleeding Disorders

Semin Thromb Hemost. 2022 Jul;48(5):529-541. doi: 10.1055/s-0041-1741571. Epub 2022 Jan 12.

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.

Publication types

  • Review

MeSH terms

  • Blood Coagulation Disorders* / complications
  • Blood Coagulation Disorders, Inherited* / complications
  • Gastrointestinal Hemorrhage / diagnosis
  • Gastrointestinal Hemorrhage / etiology
  • Gastrointestinal Hemorrhage / therapy
  • Humans
  • Retrospective Studies
  • Risk Factors