Transfer of IgA deficiency to a bone-marrow-grafted patient with aplastic anaemia.
IgA deficiency developed in a 2-year-old boy with aplastic anaemia who received a bone-marrow graft from his HLA-identical, 6-year-old, IgA-deficient sister. Southern blot analysis revealed the presence of alpha-genes in both children, thus suggesting a defect of lymphocyte stem-cell differentiation as a cause of IgA deficiency. Tissue typing showed homozygosity of HLA A1, B8, DR3, the haplotype associated with IgA deficiency in healthy people. Despite normal serum levels of IgG subclasses in both donor and recipient, both children showed a relative lack of specific IgG2 anticarbohydrate antibodies. This suggests that their IgA deficiency is part of a more fundamental aberration of immunoglobulin class and subclass distribution.
Hammarström
L
L
Lönnqvist
B
B
Ringdén
O
O
Smith
C I
CI
Wiebe
T
T
D000741:Anemia, Aplastic / Q000276:immunology / Q000628:therapy*; D000906:Antibodies / Q000276:immunology; D000954:Antigens, Surface / Q000276:immunology; D016026:Bone Marrow Transplantation; D002648:Child; D002675:Child, Preschool; D005260:Female; D006680:HLA Antigens / Q000032:analysis; D006801:Humans; D017098:IgA Deficiency; D007074:Immunoglobulin G / Q000276:immunology; D007136:Immunoglobulins / Q000032:analysis; D008297:Male; D014019:Tissue Donors
D000906:Antibodies; D000954:Antigens, Surface; D006680:HLA Antigens; D007074:Immunoglobulin G; D007136:Immunoglobulins