%0 Journal Article
%T Allogeneic Hematopoietic cell Transplantation Using Alemtuzumab in Asian Patients with Inborn Errors of Immunity.
%A Miyamoto S
%A Niizato D
%A Tomomasa D
%A Nishimura A
%A Hoshino A
%A Kamiya T
%A Isoda T
%A Takagi M
%A Kajiwara M
%A Azumi S
%A Hirabayashi S
%A Sakamoto K
%A Kishimoto K
%A Miyamura T
%A Umeda K
%A Hirose A
%A Keino D
%A Yanagimachi M
%A Kanda K
%A Sakai Y
%A Ikawa Y
%A Watanabe K
%A Tanaka K
%A Mori T
%A Ichinohe T
%A Sakaguchi H
%A Morio T
%A Kanegane H
%J J Clin Immunol
%V 44
%N 6
%D 2024 May 22
%M 38773000
%F 8.542
%R 10.1007/s10875-024-01734-5
%X Alemtuzumab is used with reduced-toxicity conditioning (RTC) in allogeneic hematopoietic cell transplantation (HCT), demonstrating efficacy and feasibility for patients with inborn errors of immunity (IEI) in Western countries; however, the clinical experience in Asian patients with IEI is limited. We retrospectively analyzed patients with IEI who underwent the first allogeneic HCT with alemtuzumab combined with RTC regimens in Japan. A total of 19 patients were included and followed up for a median of 18 months. The donors were haploidentical parents (n = 10), matched siblings (n = 2), and unrelated bone marrow donors (n = 7). Most patients received RTC regimens containing fludarabine and busulfan and were treated with 0.8 mg/kg alemtuzumab with intermediate timing. Eighteen patients survived and achieved stable engraftment, and no grade 3-4 acute graft-versus-host disease was observed. Viral infections were observed in 11 patients (58%) and 6 of them presented symptomatic. The median CD4+ T cell count was low at 6 months (241/µL) but improved at 1 year (577/µL) after HCT. Whole blood cells continued to exhibit > 80% donor type in most cases; however, 3/10 patients exhibited poor donor chimerism only among T cells and also showed undetectable levels of T-cell receptor recombination excision circles (TRECs) at 1 year post-HCT. This study demonstrated the efficacy and safety of alemtuzumab; however, patients frequently developed viral infections and slow reconstitution or low donor chimerism in T cells, emphasizing the importance of monitoring viral status and T-cell-specific chimerism. (238 < 250 words).