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자료유형
학술저널
저자정보
Hyun-Je Kim (Division of Rheumatology, Department of Internal Medicine, CHA Gumi Medical Center, CHA University, Gumi, Korea) Young Hoon Hong (Division of Rheumatology, Department of Internal Medicine, Yeungnam University College of Medicine, Daegu, Korea)
저널정보
영남대학교 의과대학 Journal of Yeungnam Medical Science Journal of Yeungnam Medical Science 제39권 제2호
발행연도
2022.4
수록면
153 - 160 (8page)
DOI
10.12701/yujm.2021.01039

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Multicentric Castleman disease (MCD) is an uncommon systemic lymphoproliferative disorder that may cause multiple organ damage. Castleman disease-associated diffuse parenchymal lung disease (DPLD) has not been well studied. A 32-year-old man was referred to our hospital for progressive generalized weakness, light-headedness, and dyspnea on exertion for more than one year. Laboratory evaluations showed profound anemia, an elevated erythrocyte sedimentation rate, and an increased C-reactive protein level with polyclonal hypergammaglobulinemia. Chest radiography, computed tomography (CT), and positron emission tomography-CT scan demonstrated diffuse lung infiltration with multiple cystic lesions and multiple lymphadenopathy. In addition to these clinical laboratory findings, bone marrow, lung, and lymph node biopsies confirmed the diagnosis of idiopathic MCD (iMCD). Siltuximab, an interleukin-6 inhibitor, and glucocorticoid therapy were initiated. The patient has been tolerating the treatment well and had no disease progression or any complications in 4 years. Herein, we report this case of human herpesvirus-8-negative iMCD-associated DPLD accompanied by multiple cystic lesions, multiple lymphadenopathy, and polyclonal hypergammaglobulinemia with elevated immunoglobulin G (IgG) and IgG4 levels. We recommend a close evaluation of MCD in cases of DPLD with hypergammaglobulinemia.

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