deamino-arginine-vasopressin and Telangiectasia--Hereditary-Hemorrhagic

Topics: Ambulatory Care; Child; Deamino Arginine Vasopressin; Epistaxis; Follow-Up Studies; Humans; Laryngoscopes; Low-Level Light Therapy; Male; Premedication; Recurrence; Retreatment; Telangiectasia, Hereditary Hemorrhagic; Treatment Outcome

A patient with hereditary hemorrhagic telangiectasia and reduced levels of von Willebrand factor had severe recurrent gastrointestinal bleeding. Treatment with either desmopressin acetate or cryoprecipitate normalized both bleeding time and kaolin-activated partial thromboplastin time. Hematochezia (five episodes) continued, however, for a week despite daily treatment with cryoprecipitate, whereas treatment with desmopressin at the onset of three later episodes of hematochezia resulted in the prompt cessation of bleeding. After an episode of life-threatening gastrointestinal bleeding, desmopressin administration allowed a total colectomy without excessive bleeding. During the 3 years after colectomy, no further gastrointestinal bleeding occurred. In the past year, however, three episodes of severe epistaxis responded promptly to intravenous infusion of desmopressin. We conclude that desmopressin may be useful in the management of bleeding in patients with hereditary hemorrhagic telangiectasia with or without von Willebrand factor deficiency.

Topics: Adult; Deamino Arginine Vasopressin; Factor VIII; Fibrinogen; Gastrointestinal Hemorrhage; Humans; Male; Recurrence; Telangiectasia, Hereditary Hemorrhagic