DDAVP normalized the bleeding time in patients with congenital platelet TxA2 receptor abnormality.
BACKGROUND
An Arg60-to-Leu mutation was found in the first cytoplasmic loop of the PLT TxA2 receptor as a new congenital PLT disorder characterized by impaired responsiveness to TxA2. However, it has not been clarified whether DDAVP is effective in correcting the bleeding time (BT) in this PLT disorder.


METHODS
DDAVP (0.4 microg/kg) was intravenously administered over 20 minutes in five patients with this PLT disorder, and template BT, PLT retention to glass beads, PLT aggregation, and a coagulation study were performed before and after the infusion of DDAVP. PLT TxA2 synthesis defects (cyclo-oxygenase deficiency, volunteers taking aspirin), thrombasthenia, and Bernard-Soulier syndrome were also included in this study.


RESULTS
The normalization of BT was found in all patients with this PLT disorder, and one of the patients successfully underwent oral surgical procedures with DDAVP as the only hemostatic agent. DDAVP was also efficacious in the TxA2 synthesis defect but not in other disorders. FVIII coagulation activity, vWF antigen, and ristocetin cofactor significantly increased in all patients after DDAVP, but no changes were seen in the PLT retention rate and PLT aggregation study after DDAVP infusion.


CONCLUSIONS
DDAVP was effective in correcting BT in patients with impaired responsiveness to TxA2 as well as impaired production of TxA2.
Fuse
Ichiro
I
Division of Blood Transfusion, Niigata University Medical Hospital, 1-754 Asahimachi, Niigata 951-8520, Japan. fuse@med.niigata-u.ac.jp
Higuchi
Wataru
W
Mito
Masao
M
Aizawa
Yoshifusa
Y
D001760:Bleeding Time; D001779:Blood Coagulation Factors / Q000032:analysis; D001791:Blood Platelet Disorders / Q000151:congenital / Q000188:drug therapy* / Q000503:physiopathology*; D001792:Blood Platelets / Q000378:metabolism*; D003894:Deamino Arginine Vasopressin / Q000009:adverse effects / Q000627:therapeutic use*; D005260:Female; D006490:Hemostatics / Q000009:adverse effects / Q000627:therapeutic use*; D006801:Humans; D008875:Middle Aged; D010509:Periodontal Cyst / Q000601:surgery; D011300:Preoperative Care; D017482:Receptors, Thromboxane / Q000378:metabolism*; D013928:Thromboxane A2 / Q000378:metabolism*; D014081:Tooth Extraction
D001779:Blood Coagulation Factors; D006490:Hemostatics; D017482:Receptors, Thromboxane; D013928:Thromboxane A2; D003894:Deamino Arginine Vasopressin