Efficacy of Lyohilized Cryoprecipitate in Controlling Epistaxis in Patients with von Willebrand Disease
Keywords:
Lyophilized cryoprecipitate, Epistaxis, von Willebrand diseaseAbstract
Abstract: A studly of the efficacy of locally-pred lyophilized cryoprecipitate (LC) was carried out in three patients with type 2A von Willebrand disease. They were two boys and one girl whose ages were 2, 12 and 9 years respectively. LC was transfused for studving pharmacokinetics of factor VIII (n-1) and controling epistaxis (n-9). The pharmacokinetic study revealed that the increment of FVIILC was 2%, vWEAg, 1% and vWERCo, 0.9% after the infusion of one unit each per kg bw. The half-life of VIIII:C was 26 h, vWf:Ag, 18 h and vWERCo, 14.7 h. In addition, the epistaxis was effectively controlled by applying anterior nasal packing and transfusing LC in a dosage of 25 units of FVIII:C per kg bw daily for 2-3 days. The bleeding time was shortened, the levels of F VII C. C. Ag and vWf.RCo were increased, and the depleted large multimers were normalized. As a result, LC infusion every 12-hour is suggested for controlling bleeding episodes in patients with von Willebrand disease. The interval can be extended to 24 hours if the local measurement is also applied to the bleeding sites. This response is based upon the clinical manifestation and levels of F VIII:C and/or vWf:RCo
Downloads
References
Werner EJ, Broxson EH, Tucker EL, et al. Prevalence of von Willebrand disease in children: a multiethnic study. J Pediatr 1993;123:893-8.
White II GC. Von Willebrand's disease. In: Jootar S. ed. Education programme of the IX Congress of the International Soceity of Haematology Asian-Pacific Division Bangkok, Thailand 1999:143-5.
Lusher JM. Systemic causes of excessive uterine bleeding. In: Jootar S, ed. Education programme of the IX Congress of the International Soceity of Haematology Asian-Pacific Division Bangkok, Thailand 1999:143-5.
Mannucci PM, Ruggeri ZM, Pareti FI, Capitanio A. 1-Deamino-8D arginine vasopressin: A new pharmacologic approach to the management of hemophiia and von Willebrand's disease. Lancet 1977;1:869-72.
Perkins HA. Correction of hemostatic defects in von Willebrand's disease. Blood 1967;30:375.
Dobrkovska A, Krzensk U, Chediak JR. Pharmacokinetics efficacy and safety of Humate-P in von Willebrand disease. Haemophilia 1998;4(suppl 3):33-9.
Chang AC, Rick ME, Ross Pierce L, Weinstein MJ. Summary of a workshop on potency and dosage of von Willebrand concentrate. Haemophilia 1998;(suppl 3):1-6.
Chantanakajornfung A, Isarangkura P, Chuansumrit A, Bunvisuthi P, Pintadit P, Nakdaeng T, Tanthien P, Hathirat P, Nuchprayoon C. Low cost production of heat treated freeze dried cryoprecipitate for treatment of hemophiliacs in Thailand. Haemophilia 199998;4:200.
Pintadit P, Isarangkura P, Chuansumrit A, Chotsupakarn S, Chantanakajornfung A. von Willbrand factor and factor VIII C levels in locally prepared lyophilized cryoprecipitate. Asian-Pacific J Allergy Immunol 1999;17:68.
Micke CH Jr, Kaneshiro MM, Marker IA, Weiner JM, Rapaport SI. The standardized normal Ivy bleeding time and its prolongation by aspirin. Blood 1969;34:205.
Hardisty RM, Macphersen JC. A one stage factor VIII (antihemophilic globuin) assay and its use on venous blood and capillary plasma. Thromb Diath Haemorrh 1962;7:215-8.
Ceijka J. Enzyme immunoassay for factor VIII related antigen. Clin Chem 1982;28:1365-8.
Brinkhous KM, LeRoy EC, Cornell WP, et al. Macroscopic studies of platelet agglutination nature of thrombocyte agglutination activity of plasma. Proc Soc Exp Biol Med 1958;8:379-83
Macfarlane DE, Stibbe J, Kirby EP, et al. A method for assaying von Willebrand factor (ristocetin cofactor). Thromb Diath Haemorth 1975;34:306-8.
Tomita Y, Harrison J, Abildgaard CF von Willebrand factor multimer analysis using a sensitive peroxidase staining method. Thromb Haemost 1989;62:781-3.
Yardumian DA, Mackie IJ, Machin SJ. Laboratory investigation of platelet function: a review of methodology. J Clin Pathol 1986;39:701-12.
Carloson M, Berntorp E, Bjorkman S, Lindvall K. Pharmacokinetic dosing in prophylactic treatment of hemophilia. Eur J Haematol 1993;51:247-52
Rodgers RPC, Levin J. A critical reappraisal of bleeding time. Semin Thromb Hemost 1990,16:1-20
Mannucci PM, Abildgaard CF, Gralnick HR, Hill FGH, Hoyer LW, Lombardi R, Nisson IM, Tuddenham E, Meyer D. Multicenter comparison of von Willebrand factor multimer sizing techniques. Report of the factor VIII and von Willebrand factor subcommittee. Thromb Haemost 1985;54:873-6.
Chuansumrit A, Isarangkura P, Chantanakajornfung A, Kuhathong K, Pintadit P, Jitpraphai C, Hathirat P. Nuchprayoon C. The efficacy and safety of lyophilized cryoprecipitate in hemophilia A. J Med Assoc Thai 1999.
Downloads
Published
Issue
Section
License
This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
