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The Korean Journal of Pathology 1982;16(3): 524-531.
이상단백혈증을 동반한 혈관 면역아구성 임파선증 1예
A Case of Angioimmunoblastic Lymphadenopathy with Dysproteinemia
A case of 53-year-old man with the diagnosis of angioimmunoblastic lymphadenopathy(AILD) is reported. This case represents a deficiency of the T-cell regulatory functions probably predisposing a nonneoplastic proliferation of the B-cell system with immunoblastic transformation of many lymphocytes and excessive production of immunoglobulins, which had an acute onset with generalized lymphadenopathy, high-grade fever, weight loss, hepatosplenomegaly, skin rash with pruritus, and malaise. Various laboratory studies showed polyclonal hypergammaglobulinemia, anemia with positive Coombs' test, leukocytosis with relative eosinophilia and lymphocytopenia. Serum immunoglobulins (IgG and IgM) were increased but IgA and complement(C3) were within normal level. Blood T-lymphocytes were markedly decreased in number. Histopathologic findings of lymph ode prior to therapy were characterized by 1) proliferation of immunoblastis, plasmacytoid immunoblasts, plasma cells and lymphocytes, b) Prominent proliferation of arborizing small vessels and c) deposit of PAS-positive amorphous material in the interstitium.
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