Idiopathic multicentric Castleman disease with pulmonary and cutaneous lesions treated with tocilizumab: A case report

World Journal of Clinical Cases
Ping-Yang HanYu-Tong Su

Abstract

Human herpes virus-8 (HHV-8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare and life-threatening disorder driven by proinflammatory cytokines, which is still poorly understood. Pulmonary parenchyma lesion is a rare condition in iMCD, which mainly manifests as lymphocytic interstitial pneumonia and is an indicator of severe iMCD. Cutaneous lesion is also very rare and mainly occurs in Asians. There have been few reports of iMCD patients with both skin and lung parenchyma involvement. We present a Chinese man who complained about a 3-year history of intermittent dry cough and a 2-year history of diffuse reddish-brown maculopapules. Laboratory examination revealed polyclonal hypergammaglobulinemia and hypercytokinemia including interleukin 6. Chest computed tomography revealed small patchy shadows with ground-glass nodules scattered in two lobes and mediastinal lymphadenopathy. The pathological result of the lymph node was consistent with the plasma cell type of Castleman disease. As serum human immunodeficiency virus test and HHV-8 staining of the lymph node were negative, the patient was finally diagnosed with HHV-8 negative iMCD. He was treated with tocilizumab at an intravenous (i.v.) dose of 8 mg/kg ever...Continue Reading

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Methods Mentioned

BETA
electrophoresis
bronchoalveolar
lavage
biopsy
biopsies
surgical resection

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Castleman Disease

Castleman disease is a rare disorder that involves an overgrowth of cells in the lymph nodes. Unicentric Castleman disease affects one lymph node, usually in the chest or abdomen. Multicentric Castleman disease affects multiple lymph nodes, commonly located in the neck, collarbone, underarm and groin areas. Discover the latest research on Castleman disease here.