Diagnosis of Glanzmann thrombasthenia by whole blood impedance analyzer (MEA) vs. light transmission aggregometry

被引:20
作者
Albanyan, A. [1 ,2 ]
Al-Musa, A. [3 ]
AlNounou, R. [4 ]
Al Zahrani, H. [3 ]
Nasr, R. [4 ]
AlJefri, A. [3 ]
Saleh, M. [4 ]
Malik, A. [1 ,2 ]
Masmali, H. [3 ]
Owaidah, T. [2 ,4 ]
机构
[1] King Saud Univ, Coll Appl Med Sci, Dept Clin Lab Sci, Riyadh, Saudi Arabia
[2] King Saud Univ, Ctr Excellence Thrombosis & Hemostasis, Riyadh, Saudi Arabia
[3] King Faisal Specialist Hosp & Res Ctr, Ctr Oncol, Riyadh 11211, Saudi Arabia
[4] King Faisal Specialist Hosp & Res Ctr, Pathol & Lab Med, Riyadh 11211, Saudi Arabia
关键词
Glanzmann's disease; platelet aggregation; multiplate; flow cytometry; PLATELET-AGGREGATION; BLEEDING DISORDERS; SPECTRUM; MUTATION;
D O I
10.1111/ijlh.12320
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
BackgroundGlanzmann 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. AimThe 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. MethodsFifteen patients with GT were assessed on MEA and LTA using arachidonic acid (ASPI: 15mm), (TRAP: 1mm), collagen (100g/mL), ADP (0.2mm), and ristocetin (Risto: 10mg/mL). Whole blood samples were collected in sodium citrate and hirudin vacuum, blood collection tubes and tested within 4h. Platelet-rich plasma was used for LTA using platelet agonists (ristocetin 1.5mg/mL) (arachidonic acid 0.5mg/mL) (ADP 2.5mg/mL) and (collagen 1mg/mL). ResultsThe platelet count and PFA-100 results were (average and SD) 31993x10(9)L and 252 +/- 34s, 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 +/- 7g, which may suggest ability to detect qualitative abnormality of GPIIb/IIIa). ConclusionThese 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.
引用
收藏
页码:503 / 508
页数:6
相关论文
共 21 条
[1]   Comparison of platelet aggregation using light transmission and multiple electrode aggregometry in Glanzmann thrombasthenia [J].
Awidi, Abdalla ;
Maqablah, Ahmad ;
Dweik, Manar ;
Bsoul, Nazzal ;
Abu-Khader, Ahmad .
PLATELETS, 2009, 20 (05) :297-301
[2]   DELIVERY OF INFANTS WITH GLANZMANN THROMBASTHENIA AND SUBSEQUENT BLOOD-TRANSFUSION REQUIREMENTS - A FOLLOW-UP OF 39 PATIENTS [J].
AWIDI, AS .
AMERICAN JOURNAL OF HEMATOLOGY, 1992, 40 (01) :1-4
[3]   PHYSICAL LINKAGE OF THE GENES FOR PLATELET MEMBRANE GLYCOPROTEIN-IIB AND GLYCOPROTEIN-IIIA [J].
BRAY, PF ;
BARSH, G ;
ROSA, JP ;
LUO, XY ;
MAGENIS, E ;
SHUMAN, MA .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1988, 85 (22) :8683-8687
[4]  
Coller BS, 1997, CIRCULATION, V95, P860
[5]   Truncation of glycoprotein (GP) IIIa (Δ 616-762) prevents complex formation with GPIIb:: Novel mutation in exon 11 of GPIIIa associated with thrombasthenia [J].
Ferrer, M ;
Tao, JM ;
Iruín, G ;
Sánchez-Ayuso, M ;
González-Rodríguez, J ;
Parrilla, R ;
González-Manchón, C .
BLOOD, 1998, 92 (12) :4712-4720
[6]   Platelet glycoprotein IIb/IIIa receptors and Glanzmann's thrombasthenia [J].
French, DL ;
Seligsohn, U .
ARTERIOSCLEROSIS THROMBOSIS AND VASCULAR BIOLOGY, 2000, 20 (03) :607-610
[7]  
GEORGE JN, 1990, BLOOD, V75, P1383
[8]  
Glanzmann E., 1918, JAHRB KINDERHEILK, V88, P113
[9]   THROMBASTHENIA - STUDIES ON 3 CASES [J].
HARDISTY, RM ;
DORMANDY, KM ;
HUTTON, RA .
BRITISH JOURNAL OF HAEMATOLOGY, 1964, 10 (03) :371-&
[10]   The PFA-100®:: a potential rapid screening tool for the assessment of platelet dysfunction [J].
Harrison, P ;
Robinson, M ;
Liesner, R ;
Khair, K ;
Cohen, H ;
Mackie, I ;
Machin, S .
CLINICAL AND LABORATORY HAEMATOLOGY, 2002, 24 (04) :225-232