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

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 plat... Mehr ...

Verfasser: Huisman, Elise J.
Holle, Nory
Schipperus, Martin
Cnossen, Marjon H.
de Haas, Masja
Porcelijn, Leendert
Zwaginga, Jaap‐Jan
Dokumenttyp: Artikel
Erscheinungsdatum: 2024
Reihe/Periodikum: Transfusion ; volume 64, issue 5, page 824-838 ; ISSN 0041-1132 1537-2995
Verlag/Hrsg.: Wiley
Sprache: Englisch
Permalink: https://search.fid-benelux.de/Record/base-27468059
Datenquelle: BASE; Originalkatalog
Powered By: BASE
Link(s) : http://dx.doi.org/10.1111/trf.17824

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.