Detection of alloimmunization in Glanzmann Thrombasthenia and Bernard-Soulier Syndrome: Data from a Brazilian Center
Gabe, Caroline; Ziza, Karen Chinoca; Durazzo, Natália; Pagani, Flavia M.; Oliveira, Valéria Brito; Conrado, Marina-C.A.V.; Dezan, Marcia R.; Mendrone Jr., Alfredo; Villaça, Paula Ribeiro; Dinardo, Carla Luana; Rocha, Vanderson.
Hematol., Transfus. Cell Ther. (Impr.)
; 45(supl.2): S101-S107, July 2023. tab, graf
Artículo
en Inglés
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LILACS-Express
| ID: biblio-1514189
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. Mainresults:
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.
Biblioteca responsable:
BR1.1
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