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. 2023 Jul;45 Suppl 2(Suppl 2):S101-S107.
doi: 10.1016/j.htct.2022.06.005. Epub 2022 Jul 20.

Detection of alloimmunization in Glanzmann Thrombasthenia and Bernard-Soulier Syndrome: Data from a Brazilian Center

Caroline Gabe  1 , Karen Chinoca Ziza  2 , Natália Durazzo  3 , Flavia M Pagani  4 , Valéria Brito Oliveira  4 , Marina-C A V Conrado  4 , Marcia R Dezan  4 , Alfredo Mendrone Jr  4 , Paula Ribeiro Villaça  2 , Carla Luana Dinardo  5 , Vanderson Rocha  6
Affiliations

Detection of alloimmunization in Glanzmann Thrombasthenia and Bernard-Soulier Syndrome: Data from a Brazilian Center

Caroline Gabe et al. Hematol Transfus Cell Ther. 2023 Jul.

Abstract

Introduction: The Glanzmann Thrombasthenia (GT) and Bernard-Soulier Syndrome (BSS) are rare hereditary disorders of platelet function. Their treatment often requires platelet transfusion, which can lead to the development of alloantibodies.

Objective: In this study, we aim to develop a strategy for alloantibody detection and to describe the frequency of alloimmunization in a patient population from a single center in southeastern Brazil.

Methods: Samples from patients with GT or BSS were tested using the Platelet Immunofluorescence Test (PIFT). If a positive result was obtained, a confirmatory step using the Monoclonal Antibody Immobilization of Platelet Antigens (MAIPA) and Luminex bead-based platelet assay (PAKLx) was executed.

Main results: Among 11 patients with GT, we detected the presence of alloantibodies in 5 using PIFT, with confirmation through MAIPA and PAKLx in 2 (1 anti-HLA and 1 anti-HPA), resulting in a frequency of 18.1%. Among 4 patients with BSS, PIFT was positive in 3, with confirmation by MAIPA and PAKLx in 1 (anti-HLA), showing a frequency of 25%. The two patients with anti-HLA antibodies exhibited a panel reactive antibody (PRA-HLA) testing greater than 97%.

Conclusion: Our study highlights the importance of identifying platelet alloimmunization in this patient population. The proposed algorithm for platelet alloantibodies detection allows resource optimization.

Keywords: Algorithms; Alloantibodies; Antigens; Bernard-Soulier syndrome; Human platelet; Platelet transfusion; Thrombasthenia.

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Conflict of interest statement

Conflicts of interest None.

Figures

Figure 1
Figure 1
Algorithm used to perform the alloantibody detection tests. Thrombastenia’ in the top box should be changed to “Thrombasthenia” Aphaeresis’ in the fourth box from the top on the left should be changed to the American spelling “Apheresis”.
See this image and copyright information in PMC

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