Diagnosis and management of gastrointestinal bleeding in patients with hereditary hemorrhagic telangiectasia.
OBJECTIVE
Our aim was to report our experience with treating GI bleeding in patients with hereditary hemorrhagic telangiectasia (HHT).


METHODS
Consecutive patients with GI bleeding referred to the Yale University Vascular Malformation Center underwent clinical evaluation and endoscopy. Hb and blood transfusion requirements for 1 yr before and after evaluation were documented. Patients with a mean Hb <or= 8 mg/dl or blood transfusion requirements >or= 12 units packed red blood cells (PRBC)/yr were defined as patients with significant bleeding. Drug therapies, including ethinyl estradiol/norethindrone, danazol, and aminocaproic acid, were prescribed on an individual patient basis.


RESULTS
The study included 43 HHT patients with a mean age of 57 yr. Endoscopy revealed telangiectases in the esophagus (1/41), stomach (33/41), duodenum (33/41), jejunum (5/9), and colon (10/32). Patients with > 20 telangiectases visualized on esophagogastroduodenoscopy had a significantly lower mean Hb of 7.9, compared with 9.4 (p = 0.007), and a trend toward higher blood transfusion requirements. Non-HHT-related causes of GI bleeding were diagnosed in four patients. During a mean follow up of 18.9 months, the group of 40 patients with HHT-related bleeding had improvements in their mean Hb and blood transfusion requirements.


CONCLUSIONS
Some HHT patients with GI bleeding improve on drug therapies, but others fail. Transfusion-dependent GI bleeding is difficult to manage, and optimal management may include both medical and endoscopic treatments.
Longacre
Anna V
AV
Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut 06520, USA.
Gross
Cary P
CP
Gallitelli
Mauro
M
Henderson
Katharine J
KJ
White
Robert I
RI
Proctor
Deborah D
DD
D000328:Adult; D000368:Aged; D005260:Female; D006471:Gastrointestinal Hemorrhage / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications*