[Thrombocytic alpha-delta-storage-pool-disease: shortening of bleeding time after infusion of 1-desamino-8-D-arginine vasopressin].
BACKGROUND
The synthetic vasopressin derivate desmopressin (1-desamino-8-D-arginine vasopressin) has been reported to shorten the bleeding time in patients with hemophilia A, von Willebrand's disease and several functional platelet disorders. In addition to substitution of platelets, vasopressin is therefore used to prevent bleeding complications.


METHODS
We report the case of a 14-year-old female patient with prolonged bleeding time due to the rare thrombocytic alpha-delta-storage-pool-disease. When normal donor platelet substitution alone was ineffective, bleeding time was normalised after infusion of desmopressin and elective wisdom-tooth extraction was performed without significant postoperative bleeding.


CONCLUSIONS
Infusion of desmopressin appears to be effective in shortening bleeding time in thrombocytic storage-pool-disease. Its use could prevent bleeding complications after trauma and surgical interventions and may possibly help to spare the need for platelet concentrates.
Kosch
A
A
Klinik und Poliklinik für Kinderheilkunde, Westfälischen Wilhelms-Universität Münster. m.a.kosch@uni-muenster.de
Kehrel
B
B
Nowak-Göttl
U
U
Häberle
J
J
Jürgens
H
H
D000293:Adolescent; D001760:Bleeding Time; D001777:Blood Coagulation / Q000187:drug effects*; D001792:Blood Platelets / Q000187:drug effects / Q000473:pathology; D003894:Deamino Arginine Vasopressin / Q000494:pharmacology / Q000627:therapeutic use*; D005260:Female; D006490:Hemostatics / Q000494:pharmacology / Q000627:therapeutic use*; D006801:Humans; D010981:Platelet Storage Pool Deficiency / Q000097:blood* / Q000150:complications / Q000188:drug therapy*; D013921:Thrombocytopenia / Q000097:blood* / Q000209:etiology; D016896:Treatment Outcome
D006490:Hemostatics; D003894:Deamino Arginine Vasopressin