DDAVP in Bernard-Soulier syndrome

THROMBOSIS RESEARCH 52; 77-78,1988 0049-3848/88 $3.00 t .OO Printed in the USA. Copyright (c) 1988 Pergamon Press plc. All rights reserved.

LETTER

DDAVP

TO THE

EDITORS-IN-CHIEF

IN BERNARD-SOULIER

SYNDROME

Michael J. Mant Division of Clinical Hematology Department of Medicine University of Alberta Edmonton, Alberta, Canada

(Received 27.5.1988; accepted in revised form 16.8.1988 by Editor-in-Chief

A.L. Copley)

reported in this journal a Cuthbert and associates recently patient with Bernard-Soulier syndrome in whom the bleeding time by infusion of DDAVP(l). I have performed similar was shortened Bernard-Soulier syndrome with studies in a patient with patient is a Caucasian female, age 24 different results. The life-long history of easy bruising, frequent years, with a menorrhagia. She has epistaxes, occasional bleeding and gum experienced prolonged bleeding following dental extractions. As result of multiple transfusions of platelets and red cells she a to random donor platelets and also h s 3 red cell is refractory b 30 x 10 /L; accurate are 25 allo-antibodies. Platelets not possible due to a tendency to platelet platelet counts are Peripheral blood smear shows marked thrombocytopenia clumping. Bleeding time is persistently > 20 with many large platelets. studies have not platelet aggregation minutes. Conventional addition of epinephrine, collagen and possible. However, been to titrated platelet rich plasma causes platelet aggregation ADP be visualised under phase contrast microscopy whereas which can factor VIIIC, vWF Plasma ristocetin does not. addition of A sister also has cofactor are normal. ristocetin antigen and Bernard-Soulier syndrome. patient The contraceptive results in

Key

Words:

has noted medication less bruising

Bernard-Soulier Desmopressin, 6-Aminocaproic

that not and

use only less

estrogen containing oral of controls her periods but also In 1984, frequent epistaxes.

Blood Platelet Syndrome, Estrogens, Cryoprecipitate, Acid. 77

Disorders,

78

DDAVP IN BERNARD-SOULIER SYNDROME

Vol. 52, No. 1

in an attempt to find therapy which might be useful for control I infused epsilon aminocaproic acid (6 g of bleeding episodes, intravenously over 30 min followed by 1 g/hour), cryoprecipitate over 30 min (10 units intravenously) and DDAVP (10 pg/m* least 7 days apart. intravenously) on separate occasions at times Bleeding were performed before, and 30 minutes and 1 hour after each There was no shortening of the bleeding infusion. time with any of these therapeutic trials. I conclude from the experience with this patient that although DDAVP be of value in some patients with potential may It is Bernard-Soulier this is not always the case. syndrome(l) possible that the absence of an effect in this patient was due to concurrent thrombocytopenia, a not unusual finding in this observation that estrogens improved bleeding disorder. The problems in this patient is similar to observations reported by Alperin (2) in patients with von Willebrand's disease.

REFERENCES

1.

CUTHBERT, RJG; LUDLAM, CA. Bernard-Soulier 649-650, 1988.

HEATHER HK; HANDA, SI; ABBOTT, I; WATSON, time in DDAVP shortens the bleeding Thrombosis Research syndrome. 49;

2.

ALPERIN, JB; and surgery in women Estrogens Willebrand's disease. Am J Med ?3;367-371, 1982.

with

von