Diffuse Lung Disease: Diffuse Lung Disease |

Castleman Disease for Ten Years Mimicking a Sarcoidosis FREE TO VIEW

Yali Zheng, MMed; Zhancheng Gao, MD
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Peking University People's Hospital, Beijing, China

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;149(4_S):A204. doi:10.1016/j.chest.2016.02.211
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SESSION TITLE: Diffuse Lung Disease

SESSION TYPE: Case Report Slide

PRESENTED ON: Saturday, April 16, 2016 at 05:15 PM - 06:15 PM

INTRODUCTION: Multicenter Castleman’s disease (MCD) is a systemic lymphoproliferative disorder characterized by generalized lymphadenopathy and associated polyclonal hyperglobulinemiar first described by Castleman in 1954 (1). A few cases show diffused pulmonary parenchymal damnification with multi-cloning plasma cellular infiltration. Herein, we report a MCD case with diffused pulmonary infiltration, whose ten years’ detailed clinical data and chest images might help to extrapolate the natural progress of pulmonary lesions.

CASE PRESENTATION: A 53 year-old female house keeper, with a history of intermittent cough and progressive exertional dyspnea of 10-year duration, was admitted for worsening dyspnea and chest tightness. She was diagnosed as “sarcoidosis” in 2002 by the pathological findings of mediastinal lymph nodes obtained using mediastinoscopy. Low dose prednisolone and intermittent cyclophosphamide were administrated since then. However, her clinical symptoms gradually improved. She needed a long term oxygen therapy to maintain her basic activities of daily living. Chest CT taken at admission revealed mediastinal and hilar adenopathy, companied with diffused pulmonary parenchymal infiltration and multiple small thin walled cysts. Lab result revealed polyclonal hyperglobulinemia, elevated erythrocyte sedimentation rate and mild anemia. Lung function test showed both obstructive and restrictive ventilator abnormalities. video-assisted thoracoscopic lung biopsy was done to explore the etiology. Histopathology and immunohistochemistry of the specimens revealed hyperplasia of the lymphoid follicles with germinal centers, plasma cell infiltration which stained positively with either anti-kappa chain or anti-lambda chain antibodies. A diagnosis of multicentric Castleman disease was finally made.

DISCUSSION: As of its diverse manifestations, multiple Castleman disease is a great mimic of both benign and malignant abnormalities. In this case, progressing diffuse parenchymal lung lesions, companied with persistent enlarged hilar and mediastinal lymph nodes and polyclonal hyperglobulinemia, which showed no response to glucocorticoids, might be the clue to the final diagnose.

CONCLUSIONS: So far, there is no data reveal the natural evolutional traits of pulmonary infiltration with MCD. Although it might be slow down by the prednisolone and cyclophosphamide treatment, her abnormal chest image findings and laboratory data for 10 years revealed the natural progress of MCD in the lung to a certain degree.

Reference #1: Castleman B, Towne VW. CASE records of the Massachusetts General Hospital Weekly Clinicopathological Exercises: Case 40011. N Engl J Med 1954;250:26-30.

DISCLOSURE: The following authors have nothing to disclose: Yali Zheng, Zhancheng Gao

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