Review and a case of rare Rosai-Dorfman disease

Abstract

Rosai-Dorfman (RDD) is a rare histiocytic syndrome with variable clinical characteristics and extranodal involvement in more than 43% of the patients. This mini-review presents an overview of the literature on Rosai-Dorfman disease. The histological features and the current understanding of the management and prognosis are discussed. A case of a 61-year-old patient with a rare diagnosis of Rosai-Dorfman disease is also presented, as well as the process of its setting and treatment. The diagnosis is set by extensive radiologic and interventional procedures, including multiple biopsies, morphological examinations, and imaging techniques. Most important for the diagnosis appeared to be the pathology showing characteristic features of Rosai-Dorfman disease in the intra-abdominal lymph nodes performed twice showing characteristic extensive fibrosis. In addition, multiple dilated sinuses were filled with macrophages and histiocytes while the immunohistochemical examination pointed very likely to RDD with increased amount of plasma cells.

Keywords

• Rosai-Dorfman disease
• histiocytosis
• abdominal lymphadenopathy