A simple, novel and robust test to diagnose type I Glanzmann thrombasthenia

Vijapurkar, Manasi; Ghosh, Kanjaksha; Shetty, Shrimati; McLane, Mary Ann; Moura-da-Silva, Ana Maria; Butera, Diego

doi:10.3324/haematol.12288

Cited by 10 publications

(6 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Vijapurkar et al recently proposed a simple one-step dot blot test using bifunctional bioengineered disintegrin/alkaline phosphatase hybrid protein ErAPv to evaluate cases of GT and normal healthy controls. The test was found highly sensitive (100%) and specific (100%) to detect type I GT, where GPIIb/GPIIIa receptors are not expressed [15]. However, in forms of GT where functionally defective GPIIb/IIIa complexes are present in the membrane, this assay was not helpful.…”

Section: Discussionmentioning

confidence: 97%

A novel ELISA for diagnosis of Glanzmann’s thrombasthenia and the heterozygote carriers

et al. 2011

View full text Add to dashboard Cite

A sensitive and specific sandwich ELISA was developed for the diagnosis of Glanzmann's thrombasthenia (GT) and the heterozygote carriers of the disease using whole blood platelets. The assay used anti-CD36 antibody to capture platelets from platelet-rich plasma which was subsequently treated with a bioengineered disintegrin/alkaline phosphatase hybrid protein specific for GP IIb/IIIa. The test allows large number of samples to be typed and can also be used on stored samples. The assay correctly diagnosed 40 normal healthy individuals, 10 GT cases, 10 heterozygotes, 3 Bernard-Soulier syndrome cases and 2 type 3 GT cases. ELISA plates were stable at room temperature up to 3 weeks without any loss of activity. This novel and simple test can be widely used for heterozygote detection besides diagnosing GT cases without using a sophisticated flow cytometer or a platelet aggregometer and has wide applicability in countries like India where many of these cases remain undiagnosed due to the lack of diagnostic facilities.

show abstract

Section: Discussionmentioning

confidence: 97%

A novel ELISA for diagnosis of Glanzmann’s thrombasthenia and the heterozygote carriers

et al. 2011

View full text Add to dashboard Cite

show abstract

“…In addition to flow cytometry in the identification of GT; Western Blot, Dot Blot, and ELISA methods have also been reported [27][28][29]. Farsinejad et al found that CD61 expression level was higher than CD41 expression level in the Western Blot analysis [27].…”

Section: Discussionmentioning

confidence: 99%

“…Vijapurkar et al emphasized that Dot Blot method could be used to diagnose type 1 GT but this method is not useful in defining other subgroups [28]. Lobo et al developed the ELISA method for detecting GT.…”

Section: Discussionmentioning

confidence: 99%

Flow cytometric analysis of platelet surface glycoproteins in the diagnosis of thirty-two Turkish patients with Glanzmann thrombasthenia: a multicenter experience

et al. 2021

View full text Add to dashboard Cite

Flow cytometric analysis of platelet surface glycoproteins in the diagnosis of thirtytwo Turkish patients with Glanzmann thrombasthenia: a multicenter experience Abstract Background/aim: Glanzmann thrombasthenia (GT) is a rare autosomal recessively inherited bleeding disorder characterized by the quantitative (type 1 and type 2) or qualitative (type 3) deficiency in platelet membrane glycoprotein (GP) IIb/IIIa (CD41a/CD61) fibrinogen receptors. In type 1, 2 and 3, CD41a/CD61 expression is 5%, 5-20% and above 20%, respectively. In this study, diagnosis of GT was confirmed and subgroups were identified in 32 Turkish patients by flow cytometry analysis.Materials and methods: CD41a/CD61 expression levels in platelet-rich plasma (PRP) obtained from peripheral venous EDTA blood samples were analyzed with a BD FACSCanto II flow cytometer (Becton Dickinson, Franklin Lakes, NJ, USA). GT subgroup analysis was performed by counting 50000 events in the BD FACSDiva Software v6.1.3 program of the instrument. Results:In the present study, in blood samples of 32 patients from 23 family with GT and 22 healthy controls, co-expression levels of CD41a and CD61 in PRP was analyzed.12 out of 23 families were consistent with type 1 GT (52.2%), 4 were consistent with type 2 GT (17.4%) and 7 were consistent with type 3 GT (30.4%). Conclusion:Especially due to consanguineous marriages, GT with various glycoprotein levels may be detected. As a result of the flow cytometry analysis of the present study with the highest GT patient population in Turkey, type 1 GT patients were the most common subgroup. In the determination of the GT subgroups; especially in the detection of type 3 GT, flow cytometry is the most sensitive glycoprotein analysis method. In 2 addition to light transmission aggregometry, CD41a/CD61 study by flow cytometer confirms diagnosis when mutation analysis cannot be performed.

show abstract

“…Are there simpler approaches to reaching a diagnosis of congenital GT in parts of the world where access to advanced techniques such as flow cytometry may be unavailable? A first step toward that goal has recently been reported by Vijapurkar and colleagues, 33 who engineered a bifunctional disintegrin/alkaline phosphatase reagent that they employed in a dot-blot procedure. Three microliters of washed platelets (containing 6 Â 10 5 platelets) were blotted onto a polyvinylidene difluoride (PVDF) membrane probed with the bifunctional reagent, and visible dots representing GPIIb/IIIa complex detected upon the addition of substrate.…”

Section: Glanzmann Thrombastheniamentioning

confidence: 99%

Glycoprotein Analysis for the Diagnostic Evaluation of Platelet Disorders

Miller

2009

Semin Thromb Hemost

View full text Add to dashboard Cite

Platelet glycoproteins subserve a wide variety of critical functions in blood platelets. Congenital deficiencies or functional abnormalities in platelet glycoproteins may produce serious bleeding disorders such as Glanzmann thrombasthenia or Bernard-Soulier syndrome. Other hematologic disorders, such as Fanconi anemia and various myelodysplastic syndromes, may also be associated with abnormalities in platelet glycoproteins. Additionally, several acquired disorders involving the major platelet glycoproteins are increasingly being recognized. The large number of techniques, now available to characterize platelet glycoprotein disorders, reflect the many advances in biochemistry, molecular analysis, flow cytometry, and, most recently, proteomics. The application of platelet glycoprotein analysis to a wide range of clinical disorders is reviewed in this article.

show abstract

A simple, novel and robust test to diagnose type I Glanzmann thrombasthenia

Cited by 10 publications

References 10 publications

A novel ELISA for diagnosis of Glanzmann’s thrombasthenia and the heterozygote carriers

A novel ELISA for diagnosis of Glanzmann’s thrombasthenia and the heterozygote carriers

Flow cytometric analysis of platelet surface glycoproteins in the diagnosis of thirty-two Turkish patients with Glanzmann thrombasthenia: a multicenter experience

Glycoprotein Analysis for the Diagnostic Evaluation of Platelet Disorders

Contact Info

Product

Resources

About