Also known as angiofollicular or giant lymph node hyperplasia, the clinical manifestations of Castleman disease (CD) are heterogeneous, ranging from asymptomatic discrete lymphadenopathy to recurrent episodes of diffuse lymphadenopathy with severe systemic symptoms.

The pathogenesis is unknown, but the bulk of evidence points toward faulty immune regulation, resulting in excessive B-lymphocyte and plasma-cell proliferation in lymphatic tissue.

There are 3 pathologic variations of Castleman disease: unicentric-hyaline vascular variant, unicentric plasma cell variant and multicentric plasma cell variant.

Patients with associated conditions, such as infection with HIV, may require special consideration. For patients with unicentric Castleman disease, surgery or irradiation, if surgery is not an option, may be curative. For those with multicentric Castleman disease, however, there are several effective, but no established standard treatment options.