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Case Report
Bone marrow transplantation for leukocyte adhesion deficiency type III: immunosuppressant dosage adjustments against severe T-cell mixed chimerism
Takashi Koike1, Eri Shigematsu1, Keisuke Otsubo1, Shigeki Ochiai1, Yoshifumi Fukuda1, Hidefumi Hiramatsu2, Hiromasa Yabe1

1Department of Pediatrics, Tokai University School of Medicine, Isehara, Japan

2Department of Pediatrics, Kinki University, Faculty of Medicine, Osaka, Japan

Keywords
leukocyte adhesion deficiency type III, unrelated donor, bone marrow transplantation, mixed chimerism, immunosuppressant dosage adjustment
Submitted: February 12, 2025
Accepted: September 8, 2025
Published online: October 31, 2025

Abstract

Allogeneic stem cell transplantation from an HLA-mismatched unrelated donor was performed for a patient with leukocyte adhesion deficiency type III with a myeloablative regimen including full-dose busulfan. Mixed chimerism with donor-derived T cells at less than 10% was observed within 4 weeks after transplantation. Repeated cycles of discontinuation and resumption of tacrolimus early after transplantation were performed with the aim of reversing the recipient-dominant T-cell chimerism. Specifically, tacrolimus was quickly tapered on day 15 and discontinued on day 20 when the recipient's chimerism increased, and resumed upon the observation of early signs of acute graft-versus-host disease, such as fever and skin rash, on day 24. This process was repeated from day 30 to day 44. All subsets, including granulocytes, T cells, and natural killer cells, attained donor chimerism of more than 90% on day 42 after transplantation and 100% at day 82 and beyond. Immunosuppressant dosage adjustments may be a treatment option for mixed chimerism after stem cell transplantation.

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Online ISSN:2432-7026