Granulocyte transfusions in patients with chronic granulomatous disease undergoing hematopoietic cell transplantation or gene therapy Journal Article


Authors: Arnold, D. E.; Chellapandian, D.; Parikh, S.; Mallhi, K.; Marsh, R. A.; Heimall, J. R.; Grossman, D.; Chitty-Lopez, M.; Murguia-Favela, L.; Gennery, A. R.; Boulad, F.; Arbuckle, E.; Cowan, M. J.; Dvorak, C. C.; Griffith, L. M.; Haddad, E.; Kohn, D. B.; Notarangelo, L. D.; Pai, S. Y.; Puck, J. M.; Pulsipher, M. A.; Torgerson, T.; Kang, E. M.; Malech, H. L.; Leiding, J. W.
Article Title: Granulocyte transfusions in patients with chronic granulomatous disease undergoing hematopoietic cell transplantation or gene therapy
Abstract: Granulocyte transfusions are sometimes used as adjunctive therapy for the treatment of infection in patients with chronic granulomatous disease (CGD). However, granulocyte transfusions can be associated with a high rate of alloimmunization, and their role in CGD patients undergoing hematopoietic cell transplantation (HCT) or gene therapy (GT) is unknown. We identified 27 patients with CGD who received granulocyte transfusions pre- (within 6 months) and/or post-HCT or GT in a retrospective survey. Twelve patients received granulocyte transfusions as a bridge to cellular therapy. Six (50%) of these patients had a complete or partial response. However, six of 10 (60%) patients for whom testing was performed developed anti-HLA antibodies, and three of the patients also had severe immune-mediated cytopenia within the first 100 days post-HCT or GT. Fifteen patients received granulocyte transfusions post-HCT only. HLA antibodies were not checked for any of these 15 patients, but there were no cases of early immune-mediated cytopenia. Out of 25 patients who underwent HCT, there were 5 (20%) cases of primary graft failure. Three of the patients with primary graft failure had received granulocyte transfusions pre-HCT and were subsequently found to have anti-HLA antibodies. In this small cohort of patients with CGD, granulocyte transfusions pre-HCT or GT were associated with high rates of alloimmunization, primary graft failure, and early severe immune-mediated cytopenia post-HCT or GT. Granulocyte transfusions post-HCT do not appear to confer an increased risk of graft failure. © 2022, This is a U.S. government work and not under copyright protection in the U.S.; foreign copyright protection may apply.
Keywords: adolescent; adult; child; clinical article; treatment response; treatment failure; retrospective studies; rituximab; bortezomib; cohort analysis; immunoglobulin; plasmapheresis; hematopoietic stem cell transplantation; retrospective study; disease severity; graft failure; gene therapy; graft versus host reaction; transplantation conditioning; cell therapy; immunosuppressive treatment; graft vs host disease; cytopenia; granulocyte; hematopoietic cell transplantation; adverse event; complication; hla antibody; granulocytes; genetic therapy; humans; human; article; chronic granulomatous disease; daratumumab; granulomatous disease, chronic; alloimmunization; granulocyte transfusions; granulocyte transfusion
Journal Title: Journal of Clinical Immunology
Volume: 42
Issue: 5
ISSN: 0271-9142
Publisher: Springer  
Date Published: 2022-07-01
Start Page: 1026
End Page: 1035
Language: English
DOI: 10.1007/s10875-022-01261-1
PUBMED: 35445907
PROVIDER: scopus
PMCID: PMC9022412
DOI/URL:
Notes: Article -- Export Date: 3 October 2022 -- Source: Scopus
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  1. Farid Boulad
    329 Boulad