Transplantation of stem cells of embryonic liver in a patient with severe combined immunodeficiency.
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.
P
P
Bührdel
M
M
Rosenkranz
H
H
Schwenke
K
K
Kühndel
V
V
Thierbach
D003586:Cytomegalovirus Infections / Q000150:complications; D005333:Fetus; D018380:Hematopoietic Stem Cell Transplantation; D006650:Histocompatibility Testing; D006801:Humans; D007105:Immune Complex Diseases / Q000209:etiology; D007075:Immunoglobulin M / Q000032:analysis; D007153:Immunologic Deficiency Syndromes / Q000235:genetics / Q000628:therapy*; D007231:Infant, Newborn; D008099:Liver / Q000166:cytology; D008213:Lymphocyte Activation; D008297:Male; D010375:Pedigree; D012131:Respiratory Insufficiency / Q000209:etiology; D012729:Sex Chromosome Aberrations / Q000235:genetics / Q000628:therapy; D013601:T-Lymphocytes / Q000276:immunology; D014960:X Chromosome; D005719:gamma-Globulins / Q000009:adverse effects
D007075:Immunoglobulin M; D005719:gamma-Globulins