異常蛋白血症을 同伴한 血管免疫芽球性 淋巴腺症 1例
A Case of Angioimmunoblastic Lymphadenopathy with Dysproteinemia
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서순팔(徐淳八)/Soon Pal Suh
박창수(朴彰洙)/정상우(鄭相愚)/유주용(柳柱容)/Chang Soo Park/Sang Woo Juhng/Joo Yong Yoo
KMID : 0357919820160030524
Abstract
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 an6 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 increase4 but IgA and complement(C3)
were within normal level. Blood T-lymphocytes were markedly decreased in number.
Histopathologic findings of lymph node prior to therapy were characterized by a)
proliferation of immunoblasts, 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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