Use of DDAVP and cryoprecipitate in mild to moderate haemophilia A and von Willebrand's disease.
DDAVP has a major therapeutic role to play in the management of moderate to mild haemophilia and vWD, particularly type I. In addition it helps to classify vWD patients and to procure more and better blood products for treatment. The addition of suitable, reliable preparations for subcutaneous and intranasal use will enable early and follow-up treatment to be self-administered. The limiting features are that FVIII:C levels are not high enough or sustained long enough for some bleeding episodes and lytic activity may occasionally be clinically significant warranting simultaneous use of antifibrinolytic agents. Side effects are minimal but caution is needed in the very young and those with vascular disease. Cryoprecipitate remains the commonest source of normal multimeric VIII:vWF. The evaluation of procedures to decrease viral transmission is incomplete as is effectiveness of different preparations in correcting the BT in vWD. Wet cryo continues to be the product most likely to correct BT in severe vWD but laboratory tests better able to predict clinical haemostasis need to be developed.
Berry
E W
EW
Haemophilia Centre, Auckland Hospital, New Zealand.
D002648:Child; D002675:Child, Preschool; D003894:Deamino Arginine Vasopressin / Q000009:adverse effects / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005169:Factor VIII / Q000627:therapeutic use*; D005260:Female; D005340:Fibrinogen / Q000627:therapeutic use*; D006467:Hemophilia A / Q000188:drug therapy*; D006801:Humans; D008297:Male; D009520:New Zealand; D014842:von Willebrand Diseases / Q000188:drug therapy*
C026912:cryoprecipitate coagulum; D005169:Factor VIII; D005340:Fibrinogen; D003894:Deamino Arginine Vasopressin