Appearance of multiple benign paraproteins during early engraftment of soy lectin T cell-depleted haploidentical bone marrow cells in severe combined immunodeficiency.
Recent advances in the prevention of graft-versus-host disease through postthymic T-cell depletion have allowed the use of haploidentical bone marrow cells for immunologic reconstitution of severe combined immunodeficiency disease. We report a male infant with severe combined immunodeficiency (with normal adenosine deaminase) who developed two IgG kappa and one IgA lambda paraproteins 7 weeks following the administration of 1.4 X 10(9) maternal bone marrow cells depleted of postthymic T cells by soy lectin agglutination and sheep erythrocyte rosetting. Serum IgG rose from 128 to 820 mg/dl, and IgA from 0 to 2400 mg/dl, peaking at 10 weeks postgrafting. By 14 weeks posttransplantation T-cell numbers and function had risen to normal (all dividing T cells had the donor karyotype) and paraprotein concentrations began to decline. These observations strongly suggest that the later-appearing T cells regulated the B-cell clones from which the paraproteins were derived. Failure of such function to appear could account for the increased incidence of B-cell lymphomas in severe combined immunodeficiency.
D001402:B-Lymphocytes / Q000276:immunology; D016026:Bone Marrow Transplantation; D006086:Graft vs Host Disease / Q000517:prevention & control; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000276:immunology / Q000378:metabolism / Q000628:therapy*; D007223:Infant; D037102:Lectins; D008212:Lymphocyte Depletion; D008297:Male; D010266:Paraproteins / Q000096:biosynthesis*; D037121:Plant Lectins; D030262:Soybean Proteins; D013601:T-Lymphocytes / Q000276:immunology; D013997:Time Factors; D010835:Phytohemagglutinins
D037102:Lectins; D010266:Paraproteins; D037121:Plant Lectins; D030262:Soybean Proteins; C028824:soybean lectin; D010835:Phytohemagglutinins