title: Diagnosis of Glanzmann thrombasthenia by whole blood impedance analyzer (MEA) vs. light transmission aggregometry.
abstract: BACKGROUND
  Glanzmann thrombasthenia (GT) is a rare inherited platelet disorder that is characterized by spontaneous or postprocedural bleeding. The diagnosis of GT depends on identifying the dysfunction of the platelets.
  
  
  OBJECTIVE
  The aim of this study was to compare a whole blood impedance Multiplate analyzer (MEA) with the standard method, light transmission aggregometry (LTA) in diagnosis of GT.
  
  
  METHODS
  Fifteen patients with GT were assessed on MEA and LTA using arachidonic acid (ASPI: 15 mm), (TRAP: 1 mm), collagen (100 μg/mL), ADP (0.2 mm), and ristocetin (Risto: 10 mg/mL). Whole blood samples were collected in sodium citrate and hirudin vacuum, blood collection tubes and tested within 4 h. Platelet-rich plasma was used for LTA using platelet agonists (ristocetin 1.5 mg/mL) (arachidonic acid 0.5 mg/mL) (ADP 2.5 mg/mL) and (collagen 1 mg/mL).
  
  
  RESULTS
  The platelet count and PFA-100 results were (average and SD) 319 ± 93 × 10(9) L and 252 ± 34 s, respectively. Flow cytometry analysis showed that all samples are positive for CD42a and CD42b, whereas 9/15 samples were negative for CD61 and CD41. The other six patients had either partial or full expression of CD61/CD41. Aggregation analysis using both methods showed that all samples had no aggregation response to any of the agonists used apart from six samples which, using only the MEA, showed minimal aggregation in response to collagen (average = 14.3 ± 7 μg, which may suggest ability to detect qualitative abnormality of GPIIb/IIIa).
  
  
  CONCLUSIONS
  These results suggest that the MEA is sensitive for the detection of Glanzmann thrombasthenia. Furthermore, MEA may also be able to differentiate between the subtypes of Glanzmann thrombasthenia.
authors.affiliation: Department of Clinical Laboratory Science, College of Applied Medical Sciences, King Saud University, Riyadh, Saudi Arabia.|Centre of Excellence in Thrombosis and Hemostasis, King Saud University, Riyadh, Saudi Arabia.
authors.forename: A
authors.initials: A
authors.lastname: Albanyan
authors.affiliation: Oncology Center, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
authors.forename: A
authors.initials: A
authors.lastname: Al-Musa
authors.affiliation: Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
authors.forename: R
authors.initials: R
authors.lastname: AlNounou
authors.affiliation: Oncology Center, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
authors.forename: H
authors.initials: H
authors.lastname: Al Zahrani
authors.affiliation: Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
authors.forename: R
authors.initials: R
authors.lastname: Nasr
authors.affiliation: Oncology Center, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
authors.forename: A
authors.initials: A
authors.lastname: AlJefri
authors.affiliation: Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
authors.forename: M
authors.initials: M
authors.lastname: Saleh
authors.affiliation: Department of Clinical Laboratory Science, College of Applied Medical Sciences, King Saud University, Riyadh, Saudi Arabia.|Centre of Excellence in Thrombosis and Hemostasis, King Saud University, Riyadh, Saudi Arabia.
authors.forename: A
authors.initials: A
authors.lastname: Malik
authors.affiliation: Oncology Center, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
authors.forename: H
authors.initials: H
authors.lastname: Masmali
authors.affiliation: Centre of Excellence in Thrombosis and Hemostasis, King Saud University, Riyadh, Saudi Arabia.|Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
authors.forename: T
authors.initials: T
authors.lastname: Owaidah
mesh_terms: D000255:Adenosine Triphosphate / Q000494:pharmacology; D000293:Adolescent; D000328:Adult; D015703:Antigens, CD / Q000235:genetics / Q000378:metabolism; D016718:Arachidonic Acid / Q000494:pharmacology; D015415:Biomarkers / Q000378:metabolism; D001792:Blood Platelets / Q000187:drug effects / Q000378:metabolism / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D003094:Collagen / Q000494:pharmacology; D017097:Electric Impedance; D015870:Gene Expression; D006801:Humans; D008027:Light; D009391:Nephelometry and Turbidimetry / Q000295:instrumentation / Q000592:standards; D010974:Platelet Aggregation / Q000187:drug effects; D010976:Platelet Count; D010979:Platelet Function Tests / Q000295:instrumentation* / Q000379:methods; D019039:Platelet Glycoprotein GPIIb-IIIa Complex / Q000235:genetics / Q000378:metabolism; D053657:Platelet-Rich Plasma / Q000166:cytology; D018179:Receptors, Thrombin / Q000737:chemistry; D012310:Ristocetin / Q000494:pharmacology; D013915:Thrombasthenia / Q000097:blood / Q000175:diagnosis*
chemical_list: D015703:Antigens, CD; D015415:Biomarkers; D019039:Platelet Glycoprotein GPIIb-IIIa Complex; D018179:Receptors, Thrombin; D012310:Ristocetin; D016718:Arachidonic Acid; D000255:Adenosine Triphosphate; D003094:Collagen
keywords: Glanzmann's disease; flow cytometry; multiplate; platelet aggregation