Erdheim-Chester Disease Presenting as a Massive Pericardial Effusion Confirmed by Bone Marrow Biopsy: A Case Report and Mini Review

Yongxu C1, Xiaomei W2, Gailin Ye1, Xiaobo W3, Lifeng W4, Lida W1, Zhongyan Li1*

1Department of Cardiology, Second Affiliated Hospital of Dalian Medical University, China
2Department of Nuclear Medicine, Second Affiliated Hospital of Dalian Medical University, China
3Department of Hematology, Second Affiliated Hospital of Dalian Medical University, China
4Department of Pathology, Second Affiliated Hospital of Dalian Medical University, China

^*Correspondance to: Zhongyan Li 

 PDF  Full Text Case Report | Open Access

Abstract:

Erdheim-Chester disease (ECD) is a non-Langerhans cell histiocytosis, which featured organs and tissues infiltrated with foam cells, causing lipoid granulomatosis and fibrosis. The incidence of ECD is extremely low, since it was first reported in 1930. When cardiac was involved predicting worse prognosis. Here, we report a case of ECD presenting as a pericardial effusion confirmed by bone biopsy. A 66-year-old man was admitted because of dyspnea and legs edema. The echocardiogram revealed moderate-large volume pericardial effusion. The PET/CT revealed pathognomonic coated aorta and highest uptake in both long bones, we performed a bone marrow biopsy. ECD was confirmed by foamy non-Langerhans histiocytes with CD68+CD1α-S100-.

Keywords:

Cite the Article:

Yongxu C, Xiaomei W, Gailin Ye, Xiaobo W, Lifeng W, Lida W, et al. Erdheim-Chester Disease Presenting as a Massive Pericardial Effusion Confirmed by Bone Marrow Biopsy: A Case Report and Mini Review. Ann Clin Case Rep. 2023; 8: 2461..