deamino-arginine-vasopressin and Melena

A patient with von Willebrand's disease had recurrent gastrointestinal bleeding from angiodysplasia, with inadequate response to von Willebrand factor substitution, medical and endoscopic treatment, and resection of affected bowel. Frequent blood transfusions were required. She started home treatment with recombinant activated factor VII (rFVIIa) at the onset of bleeding, in addition to her standard therapy. From then on, bleeds could be controlled rapidly and no more blood transfusions were needed. We conclude that rFVIIa is effective in this case of angiodysplasia and might be a therapeutic option in similar patients.

Topics: Angiodysplasia; Blood Transfusion; Combined Modality Therapy; Deamino Arginine Vasopressin; Estrogens; Factor VIIa; Factor VIII; Female; Gastrointestinal Hemorrhage; Gastroscopy; Humans; Iron; Jejunal Diseases; Melena; Middle Aged; Omeprazole; Recombinant Proteins; Recurrence; Tranexamic Acid; Treatment Outcome; von Willebrand Diseases; von Willebrand Factor

A patient with benign monoclonal IgG lambda paraproteinemia, acquired von Willebrand syndrome (AVWS), and chronic melena successfully responding to high-dose intravenous immunoglobulin (lvlg) is reported. Coagulation parameters at admission were APTT (ratio) 1.68; VIII:C 11 IU/dL; vWF:Ag 7 IU/dL:Ricof less than 3 IU/dl. RIPA was greater than 1.8 mg/ml, and bleeding time (BT) was prolonged (18 min). No evidence for an in vitro inhibitor against the VIII/vWF complex was observed. VIII/vWF measurements showed a short-lived increase after both DDAVP and Hemate P, and BT was transiently normalized. After intravenous Ig (1 g/kg for 2 days), VIII/vWF measurements, hemostatic parameters and multimeric pattern were completely corrected (VIII/C 106 IU/dl, vWF:Ag 168 IU/dl, RiCof 147 IU/dl, APTT ratio 0.89, BT 5'), with a return to pre-infusion values after 15 days. Hemoccult test became negative and packed red cell transfusions, of which 130 units were administered during the last year, were no longer required. After 18 months the patient is on maintenance treatment with repeated courses of Ig, at 3 to 4-week intervals based on VIII/vWF and BT monitoring.

Topics: Bleeding Time; Chronic Disease; Deamino Arginine Vasopressin; Dose-Response Relationship, Drug; Factor VIII; Humans; Immunoelectrophoresis; Immunoglobulins, Intravenous; Male; Melena; Middle Aged; Paraproteinemias; von Willebrand Diseases; von Willebrand Factor