title: Monitoring survival and function of transfused platelets in Bernard-Soulier syndrome by flow cytometry and a cone and plate(let) analyzer (Impact-R).
abstract: BACKGROUND
  Bernard-Soulier syndrome (BSS) patients may repeatedly require transfusion of platelets (PLTs). The hemostatic competence of transfused PLTs requires monitoring.
  
  
  METHODS
  Flow cytometry and a cone and plate(let) analyzer (Impact-R, DiaMed) were used to monitor survival and function of transfused PLTs in a 7-year-old girl with BSS undergoing surgery. Flow cytometry was applied to differentiate autologous PLTs from transfused PLTs by staining for CD42b. The Impact, which measures PLT adhesion and aggregation in response to high shear stress, was used to evaluate PLT function.
  
  
  RESULTS
  Transfused PLTs were detectable by flow cytometry for 1 week after transfusion. While the patient's PLTs did not respond to high shear stress before transfusion, a normal response was documented by the Impact on the day after transfusion and 1 week thereafter.
  
  
  CONCLUSIONS
  Transfused PLTs were detectable by flow cytometry, and their functional activity was demonstrated by the Impact.
authors.affiliation: Clinic for Blood Group Serology and Department of Pediatrics, Medical University Vienna, Vienna, Austria. simon.panzer@meduniwien.ac.at
authors.forename: Simon
authors.initials: S
authors.lastname: Panzer
authors.forename: Beate
authors.initials: B
authors.lastname: Eichelberger
authors.forename: Daniela
authors.initials: D
authors.lastname: Koren
authors.forename: Karin
authors.initials: K
authors.lastname: Kaufmann
authors.forename: Christoph
authors.initials: C
authors.lastname: Male
mesh_terms: D001606:Bernard-Soulier Syndrome / Q000150:complications / Q000503:physiopathology* / Q000628:therapy*; D001792:Blood Platelets / Q000276:immunology; D002470:Cell Survival; D002648:Child; D017147:Defibrillators, Implantable; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D008133:Long QT Syndrome / Q000150:complications / Q000601:surgery; D010973:Platelet Adhesiveness; D010974:Platelet Aggregation; D010979:Platelet Function Tests; D019038:Platelet Glycoprotein GPIb-IX Complex / Q000378:metabolism; D017713:Platelet Transfusion; D011300:Preoperative Care; D013997:Time Factors
chemical_list: D019038:Platelet Glycoprotein GPIb-IX Complex