title: [Bernard-Soulier syndrome during pregnancy: a case report].
abstract: The Bernard-Soulier syndrome (B-S s.) is rare autosomal recessive bleeding disorder. It is characterized by prolonged bleeding time, fail to agglutinate with ristocetin and a normal to decreased number of unusually large platelets whose membrane lack glycoproteins complex GP Ib/IX/V. The manifestation of the disease may differ in consecutive pregnancies of the same patient. Presently we describe 24 years old white women diagnosed with B-S s. at the age of 22. Two years later she become pregnant. During the pregnancy platelet counts ranged from 26 x 10(9)/1 to 51 x 10(9)/1, without sings of bleeding. Because of the risk of immunization against GP Ib/IX, with might lead to severe isoimmune neonatal thrombocytopenia, 4 times during pregnancy she had done MAIPA-test (monoclonal antibody immobilization of platelet antigens). Specific antibodies were not detected. Subsequent ultrasounds with biophysical profile revealed normal fetus growth. Spontaneous labour in 38th week of gestation. The use of single-donor platelets, intravenous desmopressin and tranexamic acid was found to be useful in controlling postpartum vaginal bleeding in the patient.
authors.affiliation: Instytut Hematologii i Transfuzjologii w Warszawie.
authors.forename: Izabella
authors.initials: I
authors.lastname: Kopeć
authors.forename: Krystyna
authors.initials: K
authors.lastname: Maślanko
authors.forename: Marek
authors.initials: M
authors.lastname: Litmanowicz
authors.forename: Ewa
authors.initials: E
authors.lastname: Stefańska
authors.forename: Anna
authors.initials: A
authors.lastname: Mikos
authors.forename: Krzysztof
authors.initials: K
authors.lastname: Warzocha
mesh_terms: D000328:Adult; D016824:Antigens, Human Platelet / Q000276:immunology; D001606:Bernard-Soulier Syndrome / Q000276:immunology* / Q000628:therapy*; D003894:Deamino Arginine Vasopressin / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007275:Injections, Intravenous; D017713:Platelet Transfusion; D006473:Postpartum Hemorrhage / Q000517:prevention & control; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000276:immunology* / Q000628:therapy*; D011256:Pregnancy Outcome; D014148:Tranexamic Acid / Q000008:administration & dosage; D016896:Treatment Outcome
chemical_list: D016824:Antigens, Human Platelet; D014148:Tranexamic Acid; D003894:Deamino Arginine Vasopressin