Should HLA and HPA-matched platelet transfusions for patients with Glanzmann Thrombasthenia or Bernard-Soulier syndrome be standardized care? A Dutch survey and recommendations

Elise J. Huisman*, Nory Holle, Martin Schipperus, Marjon H. Cnossen, Masja de Haas, Leendert Porcelijn, Jaap Jan Zwaginga

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

1 Citation (Scopus)
3 Downloads (Pure)

Abstract

Background: 

Glanzmann thrombasthenia (GT) and Bernard-Soulier syndrome (BSS) patients require frequent platelet transfusions and hence have an increased risk for alloimmunization against donor Human Leukocyte Antigens (HLA) when no HLA-matching is performed. Knowing that Human Platelet Antigens (HPA) are located on the platelet glycoproteins that can be absent in these patients, preventive HPA-matching may also be considered. Uniform recommendations on this topic lack in transfusion guidelines making standard practice unclear, therefore, we aimed to provide a framework for matched platelet transfusions. 

Study Design and Methods: 

We conducted a targeted literature search and a national survey of Dutch (pediatric) hematologists from July to September 2021. 

Results: 

We found 20 articles describing platelet transfusion policies in 483 GT-patients and 29 BSS-patients, both adults and children. Twenty surveys were returned for full analysis. All responders treated patients with platelet disorders, including GT (n = 36 reported) and BSS (n = 29 reported). Of respondents, 75% estimated the risk of antibody formation as “likely” for HLA and 65% for HPA. Formation of HLA antibodies was reported in 5 GT and in 5 BSS-patients, including one child. Fifteen respondents gave preventive HLA-matched platelets in elective setting (75%). Three respondents additionally matched for HPA in GT-patients (15%). Main argument for matched platelet transfusions was preventing alloimmunization to safeguard the effectivity of ‘random’ donor-platelets in acute settings. 

Conclusion: 

Elective HLA-matching for GT and BSS-patients is already conducted by most Dutch (pediatric) hematologists. HPA-matching is mainly applied when HPA-antibodies are formed. Based on the current literature and the survey, recommendations are proposed.

Original languageEnglish
Pages (from-to)824-838
Number of pages15
JournalTransfusion
Volume64
Issue number5
DOIs
Publication statusPublished - May 2024

Bibliographical note

Publisher Copyright:
© 2024 The Authors. Transfusion published by Wiley Periodicals LLC on behalf of AABB.

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