A Rare Lymphoproliferative Disease: Castleman Disease

Abstract

Castleman disease is a rare lymphoproliferative disease also known as angiofollicular lymph node hyperplasia. It is classified as hyaline vascular and plasmacytic variants histologically but characteristics of both types can coexist. Most unicentric cases of the disease are hyaline vascular while most multicentric cases are of the plasmacytic type. Although the pathogenesis is not completely understood, the role of interleukin (IL)-6 in unicentric disease and the roles of IL-6 and human herpes virus-8 in multicentric disease are well defined. Unicentric disease is typically localized and symptoms are minimal and treated locally. Multicentric disease is systemic and clinically characterized by generalized lymphadenopathy, splenomegaly, anemia, and systemic inflammatory symptoms. Systemic therapies are primarily given. Several malignant diseases including lymphomas, POEMS syndrome, follicular dendritic cell sarcomas, paraneoplastic pemphigus, Kaposi sarcoma, and amyloidosis can be associated with Castleman disease. In this paper, recent information about Castleman disease, which is a rare disease, is summarized.

CT of Castleman disease in the mediastinum

“…Castleman disease is a rare nonmalignant lymphoproliferative disorder. Clinically, CD is characterized as either multicentric CD (MCD), meaning that it involves multiple lymphatic regions, or unicentric CD (UCD), involving a single lymph node or one region of lymph nodes [5] . While UCD is clinically more predominant, MCD, which has a less favorable prognosis, is divided into 3 subgroups: human herpes virus-8-associated MCD disease, idiopathic MCD, and polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes-associated MCD.…”

“…The hyaline-vascular CD subtype is commonly diagnosed incidentally through radiological imaging modalities, with CT considered the optimal imaging tool for a proper diagnosis. UCD most commonly presents as a solitary, enlarged, well-circumscribed lymph node or localized nodal masses with an average size of 5-7 cm [ 5 , 7 , 8 ]. Lesions located in the thorax and abdomen display homogeneous high contrast enhancement, indicating hypervascularity of the mass.…”

Castleman Disease in an Older Patient With the Onset of Right Pleural Effusion

“…The HV type accounts for approximately 90% of the cases, while the PC type accounts for approximately 10% [ 3 ]. Although the HV and PC types are mainly localized, there are reports of a multicenter (MC) type that affects multiple lymph nodes throughout the body [ 4 ]. The MC type often presents histologically similar to the PC type.…”

“…A comprehensive survey of patients with MCD in Japan who were treated with tocilizumab between June 2005 and July 2011 revealed a median age of onset of 43 years [ 6 ]. CD is a relatively rare idiopathic lymphoproliferative disorder that frequently presents in the mediastinum or hilum of the lungs; however, it can also manifest in the cervical region [ 4 ].…”

Unicentric Castleman Disease: A Rare Diagnosis of Radiological and Histological Correlation

“…Morphologically the disease is classified into unicentric and multicentric Castleman disease depending upon levels of lymph nodes involvement, and according to the histopathogenetic classification it is further classified into three subtypes such as hyaline vascular Castleman disease being common one followed by plasma cell Castleman disease, and human herpesvirus 8 associated Castleman disease [ 4 ]. Fever, night sweets, weight loss and fatigue were the most common presenting complaints associated with the Castleman disease [ 5 , 6 ].…”