Successful nonsibling bone marrow transplantation in severe combined immunodeficiency

Research output: Contribution to journalJournal articleResearchpeer-review

  • K Ramsøe
  • P Skinhøj
  • V Andersen
  • P Ernst
  • V Faber
  • P Platz
  • M Thomsen
  • A Svejgaard
  • K R Eriksen
  • T Plesner
  • Morling, Niels
  • J Philip
  • S A Killmann
  • C Koch
  • N Muller-Bérat
  • K Henningsen
  • H Axelsen
Severe combined immunodeficiency (SCID) was diagnosed in a girl immediately after birth; her older brother had SCID and was successfully reconstituted by bone marrow transplantation from his uncle. She was isolated in a laminar air flow bench and decontaminated. The father differed by one HLA-A antigen but was HLA-Dw2 homozygous like the patient; his lymphocytes showed a slight response to the patient's cells in mixed lymphocyte culture (MLC). At the age of 2 1/2 months and again at 5 months, she was given a bone marrow transplant from the father. During the entire course the patient had no infections, and apart from a transient eosinophilia she had no signs of graft-versus-host reaction. Immunological reconstitution was nearly complete at 9 months of age, when she was recontaminated. One year later plasma immunoglobulin concentrations are in the low normal range (IgG and IgM) or decreased (IgA); tests of cell-mediated immunity are normal. Apart from slight upper respiratory infections, the patient has been healthy. Physical and psychological development have been normal.
Original languageEnglish
Issue number6
Pages (from-to)369-72
Number of pages3
Publication statusPublished - 1978

Bibliographical note

Keywords: Bone Marrow Transplantation; Female; HLA Antigens; Humans; Immunologic Deficiency Syndromes; Infant; Male; Pedigree; Transplantation, Homologous

ID: 18693735