Abstract
A 70-year-old woman was admitted to our hospital, in March 1981, for the evaluation of markedly elevated erythrocyte sedimentation rate (ESR) and hypergammaglobulinemia. Physical examinations revealed no abnormalities except for bilateral cervical and axillary lymphadenopathy. There was no hepatosplenomegaly. The laboratory data were: ESR 149mm/hr, Hb 11.5g/dl, WBC 5, 400/μl, platelet 133, 000/μl, and total serum protein 9.8g/dl with albumin 38.8%, α1-globulin 3.0%, α2-globulin 7.8%, β-globulin 8.0%, and γ-globulin 43.2%. The serum levels of IgG, IgA, IgM were 6, 240mg/dl, 172 mg/dl, and 96mg/dl, respectively. Plasma cells were morphologically mature and normal, while slightly increased in the bone marrow (8.6%). Histological examination of a lymph node biopsied from the left axillary area demonstrated normal structure showing marked proliferation of plasma cells around follicles. As there were no other findings suggesting liver, infectious, connective tissue, or neoplastic diseases, she was diagnosed as having idiopathic plasmacytic lymphadenopathy with polyclonal hypergammaglubilinemia (IPL). She had received no therapy until June 1985 when the serum IgG concentration tended to increase gradually. A short course of oral prednisolone therapy (25mg/day) resulted in a transient decrease in the level of serum IgG. Thereafter, she has received no special therapy because neither symptoms nor changes in the laboratory find-engs were noted. The surface marker analysis on the lymph node cells obtained from the biopsied lymph node showed that they were E-rosette positive (+) 52.8%, surface membrane (Sm)γ+39.2%, Smμ+2.9%, Smκ+ 13.5%, and Smλ+11.0%, suggesting polyclonal proliferation of IgG-producing cells. These results coresponded to the finding that Smγ, Smκ, and Smλ positive cells were demonstrated histologically on the sections of the biopsied lymph node by using the PAP stains. When peripheral mononuclear cells of this patient were cultured in vitro for 7 days with or without pokeweed mitogen, the IgG production was markedly higher than that of the normal control. The in vitro interleukin-2 production by peripheral mononuclear cells also increased. The results of peripheral lymphocyte subpopulations measured by using the flow-cytometric immunofluorescence method coupled with monoclonal antibodies were as follows. The number of OKT8+, Leu2a+/Leu15+ (suppressor), and Leu4+/HLA-DR+ (activeted T) decreased. In contrast, OKT9+, OKT10+, OKIa1+, HLA-DR+, and Leu7-/Leu11c+ (natural killer) cells increased. Surprisingly, the SmIgG+ cells did not increase in the blood. It was not explainable from this study why SmIgG+ cells decreased in the blood in spite of increased in vitro IgG production by peripheral mononuclear cells. The response to phytohemagglutinin, concanavalin-A, and pokeweed mitogen were preserved normal. Since most of the IPL cases reported previously were young adults less than 40-year-old and had usually hepatosplenomegaly, the case reported here may be clinically somewhat different from them. As the pathophysiology of IPL is not elucidated sufficiently, further clinical, histological, and immunological studies will be required.
