Immunologic studies of peripheral blood in a child with hypogammaglobulinemia. Suggested mechanism for the development of malignant B-cell lymphoma.
A case of a 7-year-old boy with common variable hypogammaglobulinemia who developed B-cell-type non-Hodgkin's lymphoma is reported. Immunologic studies of his peripheral blood before the development of lymphoma revealed: (1) although peripheral T-cell and B-cell counts were normal, serum IgG and IgA levels were remarkably reduced; (2) DNA synthesis in response to phytohemagglutinin-P (PHA), concanavalin A (Con A), and pokeweed mitogen (PWM) stimulation were decreased; (3) DNA synthesis in response to EBV was enhanced; (4) in vitro IgG production with the patient's peripheral blood lymphocytes was significantly depressed; and (5) helper and suppressor activities of the patient's T-cells did not differ from that of normal controls. Six months after the investigation systemic involvement of malignant lymphoma appeared. The lymphoma was diagnosed as a lymphoblastic diffuse one. Lymph node cell marker analysis revealed that the lymphoblasts had surface mu and kappa chain, cytoplasmic IgM, and HLA-DR antigen. Blastogenesis of B-cell series may be suppressed by a feedback mechanism with the B-cells and antibody. In the current case, impairment of such a mechanism with defect of immunoglobulin production might finally induce malignant B-lymphoid proliferation.
D000361:Agammaglobulinemia / Q000150:complications / Q000276:immunology*; D002648:Child; D004247:DNA / Q000378:metabolism; D004854:Herpesvirus 4, Human / Q000276:immunology; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis; D007074:Immunoglobulin G / Q000032:analysis / Q000096:biosynthesis; D007958:Leukocyte Count; D008214:Lymphocytes / Q000187:drug effects / Q000276:immunology / Q000528:radiation effects; D008223:Lymphoma / Q000139:chemically induced* / Q000150:complications; D008297:Male; D008934:Mitogens / Q000494:pharmacology; D014765:Viral Vaccines / Q000494:pharmacology
D007070:Immunoglobulin A; D007074:Immunoglobulin G; D008934:Mitogens; D014765:Viral Vaccines; D004247:DNA