DDAVP (desmopressin; 1‐deamino‐cys‐8‐d‐arginine‐vasopressin) treatment in children with haemophilia B

Abstract

Summary. We tested the response to desmopressin (1-deamino-cys-8-d-arginine-vasopressin; DDAVP) in four patients with haemophilia B [factor IX (F IX) at diagnosis 1´4±5%]. The activated partial thromboplastin time (aPTT) was significantly shortened in all patients. Although there was an up to 1´4-fold increase in F IX levels in three patients, maximal F IX activity remained below 10%. Much more prominent were the increases in F VIII (three-to fourfold), in von Willebrand factor antigen (VWF:Ag; 2´5-fold) and particularly in VWF collagen-binding activity (VWF:CBA; fivefold). These changes were reflected by the prophylactic efficacy of DDAVP for dental surgery. After pretesting, DDAVP could be a useful drug for reducing the need for plasma products for prevention of minor surgical bleeding in patients with mild to moderate haemophilia B.

Relationship between immunological abnormalities and paroxysmal nocturnal haemoglobinuria‐associated clones in myelodysplastic syndromes

“…In our study, DDAVP did not increase factor IX levels. However, we suggest that increased von Willebrand activity and concomitant FVIII enhancement, especially by the support of high-molecular-weight multimers, could support local haemostasis, as Ehl et al (2000) also stated that the increment of supraphysiological VWF activities might enhance the interaction between the endothelium and platelets. We also suggest that DDAVP may be as effective as in chronic renal failure .…”

Desmopressin for the treatment of haemophilia

“…However, recently Ehl et al [27] reported the clinical use of desmopressin for oral surgery in three patients …”

“…Desmopressin does not work in haemophilia B [1]. However, recently Ehl et al [27] reported the clinical use of desmopressin for oral surgery in three patients with moderate/mild haemophilia B (basal FIX:C 2.9-8 U dL )1 at the time of infusion). Despite the fact that only a 1.4-fold increase of FIX was observed after infusion, the compound proved successful in preventing bleeding.…”

How do you treat bleeding disorders with desmopressin?

“…Nevertheless, response to desmopressin has been reported in patients with mild to moderate haemophilia B, perhaps because of a generalised haemostatic effect. 25 Box 2 summarises the guidelines for the use of desmopressin in bleeding disorders.…”