Immunological reconstitution by allogeneic bone marrow transplantation in a child with the X-linked hyper-IgM syndrome

被引：16

作者：

Kawai, S ^{[1
]}

Sasahara, Y ^{[1
]}

Minegishi, M ^{[1
]}

Tsuchiya, S ^{[1
]}

Fujie, H ^{[1
]}

Ohashi, Y ^{[1
]}

Kumaki, S ^{[1
]}

Konno, T ^{[1
]}

机构：

[1] Tohoku Univ, Inst Dev Aging & Canc, Dept Paediat Oncol, Aoba Ku, Sendai, Miyagi 9808575, Japan

来源：

EUROPEAN JOURNAL OF PEDIATRICS | 1999年 / 158卷 / 05期

关键词：

X-linked hyper-IgM syndrome; CD40 ligand (CD154; bone marrow transplantation;

D O I：

10.1007/s004310051099

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

A successful transplantation of sibling marrow in a patient with the X-linked hyper-IgM syndrome is reported. Engraftment of HLA-identical marrow cells was obtained, although complicated by grade I acute graft-versus-host disease. Expression of the CD40 ligand (CD40L, CD154) by activated T-cells from the recipient remained at low levels until 10 months after the transplantation, but then normalized. The patient is now fully competent in immune function without any episodes of severe infection 24 months later. Conclusion Allogeneic bone marrow transplantation is a reasonable therapeutic option for X-linked hyper-IgM syndrome if HLA-matched family donors are available. Whether dysregulation of CD40L expression causes post-transplant immunological abnormalities remains to be clarified.

引用

页码：394 / 397

页数：4

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