TRANSFUSION 1990-Vol. M,No. 4
2. Hira PR, Husein SF. Some transfusion-induced parasitic infections in Zambia. J Hyg Epidemiol Microbiol Immunol 1979;23:436-44.
Correction of prolonged bleeding time in von Willebrand’s disease with Humate-P
To the Editor: Patients with moderately severe von Willebrand’s disease (vWD) frequently fail to respond to desmopressin acetate (DDAVP), and cryoprecipitate is the treatment of choice.’ However, mucosal bleeding requires correction of the bleeding time (BT) as well as correction of coagulation factor levels to stop hemorrhage. Even in patients with mild or moderate vWD, plasma or cryoprecipitate sometimes fails to correct the BT. Czapek2 and others3m4 have described the use of Humate-P (Behringwerke AG, Marburg, FRG), a pasteurized factor VIII concentrate that is available in limited supply in the United States, in patients whose bleeding did not respond to DDAVP. We have reportedS a case of a patient with acquired vWD, whose BT was corrected with Humate-P, at which time his prolonged gastrointestinalbleeding stopped. Recently, we treated a patient with moderately severe vWD whose bleeding did not respond to DDAVP or cryoprecipitate, or a combination of the two, but ceased promptly after the infusion of Humate-P. A 38-year-old man with known, moderately severe vWD was admitted to the hospital with significant blood loss from prolonged epistaxis that was unresponsive to DDAVP infusion. When tested 2 years previously, the patient’s factor VIII coagulant (FVIIkC) and von Willebrand factor antigen (vWF:Ag) had been found to respond to DDAVP, although the von Willebrand factor (vWF) and BT showed little change (Table l). On this occasion, cryoprecipitate was administered (10 bags/ 12 hours), and, despite normalization of FVII1:C and vWF, the bleeding time remained prolonged. Because of continued bleeding that required nasal packing, the patient also received an infusion of DDAVP after a dose of cryoprecipitate (Day 2). Again, despite normalization of levels, the BT remained more than 15 minutes. Attempted removal of the nasal packing resulted in recurrent hemorrhage that produced a severe anemia (hematocrit, 15). The patient required both anterior and posterior packing. The packing was left in place for several days, during which the patient received cryoprecipitate every 12 hours. He was then given an infusion of Humate-P (2600 U = 35 U/ kg) on Day 9 to raise the FVII1:C and vWF to 200 percent. Immediately after this, the BT corrected to 9 minutes and the packing was removed without incident. The patient achieved
Table 1. Patient response to DDAVP, cryoprecipitafe, and Humate-P Treatment 9 Baseline (1987) After DDAVP (1987) 9.5 Cryoprecipitate alone (Day 2) > 15 Cryoprecipitate + DDAVP >15 Cryoprecipitate alone (Day 9) > 15 Humate-P 9
‘Bleeding time. tFactor Vlll coagulant. won Willebrand factor. Bvon Willebrand antigen.
381
LETTERS TO THE EDITOR
17 99 85 87 85 102
el0 17 44 77 19 260
2. Hira PR, Husein SF. Some transfusion-induced parasitic infections in Zambia. J Hyg Epidemiol Microbiol Immunol 1979;23:436-44.
Correction of prolonged bleeding time in von Willebrand’s disease with Humate-P
To the Editor: Patients with moderately severe von Willebrand’s disease (vWD) frequently fail to respond to desmopressin acetate (DDAVP), and cryoprecipitate is the treatment of choice.’ However, mucosal bleeding requires correction of the bleeding time (BT) as well as correction of coagulation factor levels to stop hemorrhage. Even in patients with mild or moderate vWD, plasma or cryoprecipitate sometimes fails to correct the BT. Czapek2 and others3m4 have described the use of Humate-P (Behringwerke AG, Marburg, FRG), a pasteurized factor VIII concentrate that is available in limited supply in the United States, in patients whose bleeding did not respond to DDAVP. We have reportedS a case of a patient with acquired vWD, whose BT was corrected with Humate-P, at which time his prolonged gastrointestinalbleeding stopped. Recently, we treated a patient with moderately severe vWD whose bleeding did not respond to DDAVP or cryoprecipitate, or a combination of the two, but ceased promptly after the infusion of Humate-P. A 38-year-old man with known, moderately severe vWD was admitted to the hospital with significant blood loss from prolonged epistaxis that was unresponsive to DDAVP infusion. When tested 2 years previously, the patient’s factor VIII coagulant (FVIIkC) and von Willebrand factor antigen (vWF:Ag) had been found to respond to DDAVP, although the von Willebrand factor (vWF) and BT showed little change (Table l). On this occasion, cryoprecipitate was administered (10 bags/ 12 hours), and, despite normalization of FVII1:C and vWF, the bleeding time remained prolonged. Because of continued bleeding that required nasal packing, the patient also received an infusion of DDAVP after a dose of cryoprecipitate (Day 2). Again, despite normalization of levels, the BT remained more than 15 minutes. Attempted removal of the nasal packing resulted in recurrent hemorrhage that produced a severe anemia (hematocrit, 15). The patient required both anterior and posterior packing. The packing was left in place for several days, during which the patient received cryoprecipitate every 12 hours. He was then given an infusion of Humate-P (2600 U = 35 U/ kg) on Day 9 to raise the FVII1:C and vWF to 200 percent. Immediately after this, the BT corrected to 9 minutes and the packing was removed without incident. The patient achieved
Table 1. Patient response to DDAVP, cryoprecipitafe, and Humate-P Treatment 9 Baseline (1987) After DDAVP (1987) 9.5 Cryoprecipitate alone (Day 2) > 15 Cryoprecipitate + DDAVP >15 Cryoprecipitate alone (Day 9) > 15 Humate-P 9
‘Bleeding time. tFactor Vlll coagulant. won Willebrand factor. Bvon Willebrand antigen.
381
LETTERS TO THE EDITOR
17 99 85 87 85 102
el0 17 44 77 19 260
