一名患有X连锁高IgM综合征儿童通过异基因骨髓移植实现免疫重建。

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

作者信息

Kawai S, Sasahara Y, Minegishi M, Tsuchiya S, Fujie H, Ohashi Y, Kumaki S, Konno T

机构信息

Department of Paediatric Oncology, Institute of Development, Aging and Cancer, Tohoku University, Sendai, Japan.

出版信息

Eur J Pediatr. 1999 May;158(5):394-7. doi: 10.1007/s004310051099.

DOI:10.1007/s004310051099

PMID:10333122

Abstract

UNLABELLED

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.

摘要

未标注

报道了一名患有X连锁高IgM综合征的患者成功接受同胞骨髓移植的病例。获得了HLA匹配的骨髓细胞植入，尽管并发了I级急性移植物抗宿主病。受体活化T细胞的CD40配体（CD40L，CD154）表达在移植后10个月一直处于低水平，但随后恢复正常。24个月后，患者现在免疫功能完全正常，没有任何严重感染发作。