Transplantation of stem cells of embryonic liver in a patient with severe combined immunodeficiency

Acta Paediatr Hung. 1985;26(3):233-40.

Abstract

A patient had severe combined immunodeficiency syndrome and X-chromosomal recessive heredity. Since the parents and siblings were not suitable as HLA-compatible bone marrow donors, stem cells from embryonic liver were transplanted intravenously in 3 stages (6 X 10(6); 3.5 X 10(6), and 9 X 10(7]. Transplantation was tolerated well; there were no signs of a graft-versus-host reaction. Examination of the immunological condition after transplantation showed evidence of T-cell reconstitution, immunohistochemistry revealed beginning immune globulin production. The child died at the age of 5 months due to respiratory failure.

Publication types

  • Case Reports

MeSH terms

  • Cytomegalovirus Infections / complications
  • Fetus
  • Hematopoietic Stem Cell Transplantation*
  • Histocompatibility Testing
  • Humans
  • Immune Complex Diseases / etiology
  • Immunoglobulin M / analysis
  • Immunologic Deficiency Syndromes / genetics
  • Immunologic Deficiency Syndromes / therapy*
  • Infant, Newborn
  • Liver / cytology
  • Lymphocyte Activation
  • Male
  • Pedigree
  • Respiratory Insufficiency / etiology
  • Sex Chromosome Aberrations / genetics
  • Sex Chromosome Aberrations / therapy
  • T-Lymphocytes / immunology
  • X Chromosome
  • gamma-Globulins / adverse effects

Substances

  • Immunoglobulin M
  • gamma-Globulins