Preoperative blood transfusion for gynecological operation of a patient with Bernard-Soulier syndrome: Case report

Bernard-Soulier syndrome belongs to congenital thrombocytopathic platelet disorders. There is a change of the structure of the glycoprotein in platelet membrane, causing the impair of platelet adherence on the blood vessel wall. This syndrome is clinically manifested by spontaneous bleeding in the skin and mucosa. The prognosis is usually good with an adequate support, but serious bleeding episodes occur during menstruation, trauma or surgery intervention. Treatment of bleeding or prophylaxis during surgical intervention is usually based upon platelet transfusion and the use of antifibrinolitic drugs. The object of case report is the significance of the right and an adequate preparation for the operational treatment: Mrs 42 year old, with diagnosis: Bernard-Soulier thrombocytopathia. Iron deficiency anemia. Status post operationem cystis ovarii sinistri. Admitted to the Clinic of gynaecology and obstetrics 'Narodni front' for operative treatment. The menstrual cycle is on 28 days, duration 7 days. From juvenile period there were reports of episodes of bleeding with thrombocytopathia. In prepartal period transfused with few doses of platelet. All dental interventions followed with bleeding, done with 6 doses of platelet concentrate. The history of operation of a cyst with a diagnosis: Cysta ovarii lateralis dextri torquata in 2005. The operation followed with pre-operative use of 15 doses of platelet concentrate, 2 units of fresh frosen plasm and 3 units of deplasmatic erythrocytes. There was a report of adverse reaction due to plasm transfusion and erythrocytes as a hypersensitive reaction, but during operation, there was no bigger post-operative bleeding. In following 2 years, the patient was hospitalized few times because of seriuos menometrorrhagia, and conservativly treated with iron preparations, with a difficult tolerating. Anamnesis: allergy to preparation of salicylate, ranitidin, diclofenac and tranexamic acid. In last hospitalization, the patient was admitted because of a large bleeding. Haematological parameters: Hgb 63 g/L, Rbc 2,61 x 1012/l, MCV 76fL, Plt 22 x 109/l. Biochemical parameters in referential values. Global tests of haemostasis preoperativly: PT 13,4s (9-12,6s), INR 1,02, APTT 20,7s (24-35s), fib 2,08 g/L (1,69-5,15 g/L), TT 18,9s (18-25s), 3 doses of deplasmatic erythrocytes and 2 x 7 doses of platelet concentrate with preoperativly used of methylprednisone (80 mg). Operation: Hysterectomia totalis abdominalis classica cum adnexectomiam lateralis dextri. Pre-operative and post-operative period regular. Therapy: antibiotics, ana