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Immunologic studies of peripheral blood in a child with hypogammaglobulinemia. Suggested mechanism for the development of malignant B-cell lymphoma

✍ Scribed by Hiroyuki Tsuchiya; Shigenori Higuchi; Tsunehiro Kuwahara; Ichiro Matsuda; Hiroaki Mitsuya; Kazunari Yamaguchi


Publisher
John Wiley and Sons
Year
1984
Tongue
English
Weight
505 KB
Volume
53
Category
Article
ISSN
0008-543X

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✦ Synopsis


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 'I-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 IgC; production with the patient's peripheral blood lymphocytes was significantly depressed; and ( 5 ) helper and suppressor activities of the patient's 1 -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 and K 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 ofsuch a mechanism with defect of immunoglobulin production might finally induce malignant B-lymphoid proliferation.

Cuncer 53:1492-1497, 1984.

T IS WELL KNOWN that persons with primary abnor-I malities of the immune system develop more cancers than the general population. In the case of children with such abnormalities, non-Hodgkin's lymphomas and lymphoid leukemias are major types of malignancies.' Detailed investigation may clarify the relationship between these two disorders. In this article. we report on a 7-year-old boy with common variable hypogammaglobulinemia (CVH) who developed B-cell-type lymphoblastic diffuse lymphoma.