title: Bernard-Soulier-like functional platelet defect in myelodysplastic syndrome and in acute myeloblastic leukemia associated with trilineage myelodysplasia.
abstract: Platelet function was studied in a child with myelodysplastic syndrome (MDS: refractory anemia with an excess of blasts) and a child with acute myeloblastic leukemia (AML-M6) associated with trilineage myelodysplasia (TMDS). An acquired Bernard-Soulier-like platelet defect was considered in both patients with the findings of prolonged bleeding time and abnormally large platelets that failed to aggregate in response to ristocetin. In contrast to findings in von Willebrand's disease, the abnormal response of platelets to ristocetin could not be corrected by the addition of normal flesh plasma. The detection of abnormal platelet aggregation response to ristocetin may be a useful diagnostic finding for clonal disorders causing impaired platelet function in MDS and coexistent TMDS associated with AML. Further studies of ristocetin-induced platelet aggregation in a large number of these patients are required.
authors.affiliation: Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.
authors.forename: G
authors.initials: G
authors.lastname: Hiçsönmez
authors.forename: F
authors.initials: F
authors.lastname: Gümrük
authors.forename: M
authors.initials: M
authors.lastname: Cetin
authors.forename: N
authors.initials: N
authors.lastname: Ozbek
authors.forename: M
authors.initials: M
authors.lastname: Tuncer
authors.forename: T
authors.initials: T
authors.lastname: Gürsel
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D000754:Anemia, Refractory, with Excess of Blasts / Q000097:blood / Q000150:complications; D001606:Bernard-Soulier Syndrome; D001791:Blood Platelet Disorders / Q000209:etiology*; D002675:Child, Preschool; D005260:Female; D006801:Humans; D015470:Leukemia, Myeloid, Acute / Q000097:blood / Q000150:complications*; D009190:Myelodysplastic Syndromes / Q000097:blood / Q000150:complications*; D010974:Platelet Aggregation