title: Fatal lymphoma after transplantation of cultured thymus in children with combined immunodeficiency disease.
abstract: A fatal, widespread, polyclonal, B-cell immunoblastic lymphoproliferative disorder developed in three children with combined immunodeficiency shortly after intra-abdominal transplantation of cultured thymus epithelium for immunoreconstitution. All three had surface immunoglobulin-bearing cells (15 to 20 per cent) in the peripheral blood before transplantation and polyclonally elevated immunoglobulins afterward. Abnormal immunoregulation was demonstrated by a lack of concanavalin A-induced suppressor-cell activity in mixed leukocyte culture in all three patients before transplantation and in two afterward. We suggest that the transplant acted as a promoter through immunostimulation or production of promoter factors, and that excessive polyclonal B-cell proliferation resulted because of inadequate immunoregulatory mechanisms. Although this complication occurred in only three of 30 patients with various forms of immunodeficiency treated with cultured thymus, these cases illustrate a potential problem in immunoreconstitution of combined immunodeficiency disorders.
mesh_terms: D001402:B-Lymphocytes / Q000276:immunology; D002455:Cell Division; D002675:Child, Preschool; D004848:Epithelium / Q000637:transplantation; D005260:Female; D006801:Humans; D007119:Immunoblastic Lymphadenopathy / Q000209:etiology* / Q000276:immunology; D007136:Immunoglobulins / Q000032:analysis; D007153:Immunologic Deficiency Syndromes / Q000276:immunology / Q000628:therapy*; D007223:Infant; D008198:Lymph Nodes / Q000473:pathology; D007959:Lymphocyte Culture Test, Mixed; D008223:Lymphoma / Q000209:etiology* / Q000276:immunology; D008297:Male; D009924:Organ Culture Techniques; D011947:Receptors, Antigen, B-Cell / Q000032:analysis; D013601:T-Lymphocytes / Q000276:immunology; D013950:Thymus Gland / Q000637:transplantation*; D014184:Transplantation, Homologous
chemical_list: D007136:Immunoglobulins; D011947:Receptors, Antigen, B-Cell