KMID : 1039420180520030183
|
|
Journal of Pathology and Translational Medicine 2018 Volume.52 No. 3 p.183 ~ p.190
|
|
Erdheim-Chester Disease Involving Lymph Nodes and Liver Clinically Mimicking Lymphoma: A Case Report
|
|
Sung Yeoun-Eun
Lee Yoon-Seo Lee Ji-Eun Lee Kyo-Young
|
|
Abstract
|
|
|
Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis and multisystem disease. First described in 1930, there are no more than 750 cases reported. The etiology remains unknown, but a majority of cases of ECD and Langerhans cell histiocytosis were found to have clonal mutations involving genes of the mitogen-activated protein kinase pathway. We recently encountered a 53-year-old male patient with extensive ECD involving the systemic lymph nodes, pleura, liver, and long bones clinically mimicking malignant lymphoma. Biopsies were performed at multiple sites, including a pleural mass, an external iliac lymph node, bone marrow, and the liver. Based on histopathological and immunohistochemical findings of positivity for CD68 and negativity for CD1a and S-100, the patient was diagnosed with ECD. Interferon-¥á was administered as the first-line treatment, but the patient rapidly progressed to hepatic failure after 2 months of treatment. We report this rare case of ECD clinically mimicking malignant lymphoma and diagnosed by careful pathological review.
|
|
KEYWORD
|
|
Erdheim-Chester disease, Lymph nodes, Vertebrae, Pleura, Liver
|
|
FullTexts / Linksout information
|
|
|
|
Listed journal information
|
|
|
|