Monoclonal immunoglobulin-secreting lymphoma in a patient with severe combined immunodeficiency disease.
A 3.5 year old boy with X-linked severe combined immunodeficiency disease (SCID), who had been in laminar flow isolation throughout his life, developed a B cell tumour producing up to 3008 mg/dl of an IgM kappa paraprotein 1 month after infusion of both liver and thymus cells from a fetal donor and 6 months after the last of six fetal liver cell infusions given over a 3 year period. Pretransplant studies revealed a high percentage of circulating B lymphocytes. HLA typing suggests that the tumour was of host origin.
D000375:Aging; D001402:B-Lymphocytes; D002675:Child, Preschool; D006680:HLA Antigens / Q000032:analysis; D006801:Humans; D007075:Immunoglobulin M / Q000096:biosynthesis*; D007145:Immunoglobulin kappa-Chains / Q000096:biosynthesis; D007153:Immunologic Deficiency Syndromes / Q000150:complications / Q000276:immunology* / Q000628:therapy; D016031:Liver Transplantation; D008213:Lymphocyte Activation; D008223:Lymphoma / Q000150:complications / Q000276:immunology* / Q000648:ultrastructure; D008297:Male; D008854:Microscopy, Electron; D013950:Thymus Gland / Q000637:transplantation
D006680:HLA Antigens; D007075:Immunoglobulin M; D007145:Immunoglobulin kappa-Chains