Immunologic reconstitution following Bone Marrow Transplantation (BMT) for X-linked hyper IgM (XHIM) syndrome

J. E. Duplantier, N. K. Day, Robert Nelson, R. Hitchock, H. D. Ochs, K. Seyama, S. Haraguchi, M. Klemperer, R. A. Good

Research output: Contribution to journalArticle

Abstract

We have performed sequential immunologic evaluation of a child with XHIM following fully-matched sibling BMT at 8 mo. The child is now 19 mo. post-BMT and remains well engrafted. No GVHD has developed. Serum immunoglobulins are normal, activated T cells express CD40 ligand (CD40L), and analyses of DNA obtained from PBLs, cultured T & B cells reveal normal CD40L sequences. Phi X 174 immunization given 9 mo. after BMT resulted in a normal primary response. Following a secondary immunization, the patient responded with amplification although titers were decreased and class switching was impaired (7% vs 49% in controls). BMT has the potential to cure XHIM not only in terms of susceptibility to infection, but also with respect to other potentially fatal complications such as malignancies. BMT appears to be the treatment of choice for XHIM if a fully-matched sibling donor is available. The success of BMT, especially if only a matched unrelated donor is available, may depend on the age of the patient.

Original languageEnglish (US)
JournalFASEB Journal
Volume12
Issue number5
StatePublished - Mar 20 1998
Externally publishedYes

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Type 1 Hyper-IgM Immunodeficiency Syndrome
bone marrow transplant
Bone Marrow Transplantation
Immunoglobulin M
Bone
Immunization
CD40 Ligand
Siblings
Immunoglobulin Class Switching
Secondary Immunization
Unrelated Donors
T-cells
immunoglobulins
B-lymphocytes
Amplification
Immunoglobulins
immunization
B-Lymphocytes
T-lymphocytes
Cells

ASJC Scopus subject areas

  • Agricultural and Biological Sciences (miscellaneous)
  • Biochemistry, Genetics and Molecular Biology(all)
  • Biochemistry
  • Cell Biology

Cite this

Duplantier, J. E., Day, N. K., Nelson, R., Hitchock, R., Ochs, H. D., Seyama, K., ... Good, R. A. (1998). Immunologic reconstitution following Bone Marrow Transplantation (BMT) for X-linked hyper IgM (XHIM) syndrome. FASEB Journal, 12(5).

Immunologic reconstitution following Bone Marrow Transplantation (BMT) for X-linked hyper IgM (XHIM) syndrome. / Duplantier, J. E.; Day, N. K.; Nelson, Robert; Hitchock, R.; Ochs, H. D.; Seyama, K.; Haraguchi, S.; Klemperer, M.; Good, R. A.

In: FASEB Journal, Vol. 12, No. 5, 20.03.1998.

Research output: Contribution to journalArticle

Duplantier, JE, Day, NK, Nelson, R, Hitchock, R, Ochs, HD, Seyama, K, Haraguchi, S, Klemperer, M & Good, RA 1998, 'Immunologic reconstitution following Bone Marrow Transplantation (BMT) for X-linked hyper IgM (XHIM) syndrome', FASEB Journal, vol. 12, no. 5.
Duplantier, J. E. ; Day, N. K. ; Nelson, Robert ; Hitchock, R. ; Ochs, H. D. ; Seyama, K. ; Haraguchi, S. ; Klemperer, M. ; Good, R. A. / Immunologic reconstitution following Bone Marrow Transplantation (BMT) for X-linked hyper IgM (XHIM) syndrome. In: FASEB Journal. 1998 ; Vol. 12, No. 5.
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