T-cell-depleted CD34+ cell transplantation from an HLA-mismatched donor in a low-birthweight infant with X-linked severe combined immunodeficiency

Akihiko Nomura, Hidetoshi Takada, Shoichi Ohga, Naoto Ishii, Toshiro Inoue, Toshiro Hara

Research output: Contribution to journalArticle

Abstract

The best strategy of hematopoietic stem cell (HSC) transplantation for low-birthweight (LBW) infants with severe combined immunodeficiency (SCID) remains to be determined. To avoid the toxicity of drugs used for the transplantation and the risk of graft-versus-host disease (GVHD), the authors performed allogeneic bone marrow HSC transplantation with a combination of CD34 selection and T-cell depletion in a LBW infant with X-linked SCID. The authors analyzed the process of T-cell reconstitution after the transplantation in this patient. The patient was born at 30 weeks and 2 days' gestational age via cesarean section. He was diagnosed as having SCID at birth. The patient received a transplant of 1 million CD34+ cells/kg body weight. Immunologic reconstitution was investigated by means of phenotypic analysis of T cells and genetic analysis of coding joint T-cell receptor rearrangement excision circle expression. Increases in donor-derived NK cells and T cells were observed 2 and 3 months after the transplantation, respectively. The patient had no infectious complications or GVHD despite the presence of SCID and prematurity-associated immunodeficiency. Analysis of T-cell regeneration pathways revealed that T cells reconstituted mainly via the thymus-dependent pathway. T-cell-depleted CD34+ cell transplantation would be a safe and useful therapy for LBW infants with SCID.

Original languageEnglish
Pages (from-to)80-84
Number of pages5
JournalJournal of Pediatric Hematology/Oncology
Volume27
Issue number2
DOIs
Publication statusPublished - Feb 1 2005

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X-Linked Combined Immunodeficiency Diseases
Cell Transplantation
Severe Combined Immunodeficiency
Tissue Donors
T-Lymphocytes
Transplantation
Hematopoietic Stem Cell Transplantation
Graft vs Host Disease
T-Cell Antigen Receptor
Drug-Related Side Effects and Adverse Reactions
Cesarean Section
Natural Killer Cells
Thymus Gland
Gestational Age
Regeneration
Joints
Bone Marrow
Body Weight
Parturition
Transplants

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

Cite this

T-cell-depleted CD34+ cell transplantation from an HLA-mismatched donor in a low-birthweight infant with X-linked severe combined immunodeficiency. / Nomura, Akihiko; Takada, Hidetoshi; Ohga, Shoichi; Ishii, Naoto; Inoue, Toshiro; Hara, Toshiro.

In: Journal of Pediatric Hematology/Oncology, Vol. 27, No. 2, 01.02.2005, p. 80-84.

Research output: Contribution to journalArticle

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