Castleman�s Disease - Clinical, Histological and Therapeutic Features

Abstract

Castleman�s disease (CD) is a rare and benign lymphoproliferative pathology, characterized by lymphoid tissue hyperplasia, process that can occur at any site of the lymphoid chain. The purpose of this paper is to review the existing data regarding Castleman�s disease etiopathogenesis and treatment. Considering the extent of the lymphoid tissue involvement Castleman�s disease can be classified as unicentric (UCD) and multicentric (MCD). Another classification of this pathology is based on the histopathological features: hyaline vascular CD (90% of cases), plasma cell CD (less than 10%) and mixed cell type. Patients with UCD have good prognosis, the gold standard treatment being complete surgical excision. The multicentric type in contrast to UCD has a worse prognosis and associates the risk of evolving to lymphoma. Over the years different therapeutic strategies have been applied in the management of multicentric Castleman�s disease: glucocorticoids, chemotherapy, antiviral agents and monoclonal antibodies that target CD (cluster of differentiation) 20, interleukin -6 (IL-6) and IL-6 receptors. Castleman�s disease is a rare and complex pathology, whose etiopathogenesis is still incompletely elucidated. In the past few years the overall survival and progression free survival has significantly increased, due to different therapeutic options that have emerged, options that have constantly offered better and better results. Further investigation regarding the chemical interactions between different receptors and therapeutic molecules, understanding the mechanism of action and the potential benefits of each therapeutic agent may prove useful in clinical practice for treating CD.