AI Article Synopsis

  • Glanzmann thrombasthenia (GT) and Bernard-Soulier syndrome (BSS) are conditions where patients often need platelet transfusions, which can cause problems if they react to donor blood types.
  • Doctors in the Netherlands were surveyed to find out how they handle platelet matches, and many believe it's important to prevent issues with antibodies from forming.
  • The study suggests that most pediatric doctors are already matching blood types when giving transfusions to these patients, and they recommend more matching based on some guidelines.

Article 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.

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Source
http://dx.doi.org/10.1111/trf.17824DOI Listing

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