Granulocyte Transfusions in Patients with Chronic Granulomatous Disease Undergoing Hematopoietic Cell Transplantation or Gene Therapy

Danielle E. Arnold, Deepak Chellapandian, Suhag Parikh, Kanwaldeep Mallhi, Rebecca A. Marsh, Jennifer R. Heimall, Debra Grossman, Maria Chitty-Lopez, Luis Murguia-Favela, Andrew R. Gennery, Farid Boulad, Erin Arbuckle, Morton J. Cowan, Christopher C. Dvorak, Linda M. Griffith, Elie Haddad, Donald B. Kohn, Luigi D. Notarangelo, Sung Yun Pai, Jennifer M. PuckMichael A. Pulsipher, Troy Torgerson, Elizabeth M. Kang, Harry L. Malech, Jennifer W. Leiding

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish (US)
Pages (from-to)1026-1035
Number of pages10
JournalJournal of Clinical Immunology
Volume42
Issue number5
DOIs
StatePublished - Jul 2022

Keywords

  • Alloimmunization
  • Chronic granulomatous disease
  • Graft failure
  • Granulocyte transfusions
  • Hematopoietic cell transplantation

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

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