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Unicentric Castleman's disease

Jennifer R Brown, MD, PhD
Jon C Aster, MD
Nikhil C Munshi, MD
Section Editor
Arnold S Freedman, MD
Deputy Editor
Rebecca F Connor, MD


Castleman's disease (CD, angiofollicular lymph node hyperplasia) is a heterogenous group of lymphoproliferative disorders associated in a subset of cases with the human immunodeficiency virus (HIV) and human herpesvirus 8 (HHV-8). CD comprises at least two distinct diseases (unicentric and multicentric) with very different prognoses. It is also associated with a number of malignancies, including Kaposi sarcoma, non-Hodgkin lymphoma, Hodgkin lymphoma, and POEMS syndrome.

CD was first described in 1956 by Benjamin Castleman and colleagues, who identified a series of patients with solitary hyperplastic mediastinal lymph nodes containing small, hyalinized follicles and a marked interfollicular vascular proliferation (hyaline vascular variant of CD) [1]. The same investigators later identified lymph nodes with a similar vascular proliferation associated with large hyperplastic germinal centers and sheets of interfollicular plasma cells (plasma cell variant of CD). Hyalinized follicles were present in some, but not all, such cases.

All of the patients described in these early papers had localized disease, which is now termed unicentric Castleman's disease (UCD). UCD is associated with systemic symptoms in a subset of cases.

In contrast, multicentric Castleman's disease (MCD) is a systemic disease with generalized peripheral lymphadenopathy, hepatosplenomegaly, frequent fevers, and night sweats that is more frequently associated with the plasma cell variant. Unlike UCD, MCD is strongly associated with immunosuppression and HHV-8 infection.

This topic review will discuss the epidemiology, pathogenesis, clinical and pathologic features, diagnosis, and treatment of UCD. MCD and a review of diseases associated with HHV-8 infection and the virology of HHV-8 are presented separately. (See "Multicentric Castleman's disease" and "Disease associations of human herpesvirus 8 infection" and "Virology, epidemiology, and transmission of human herpesvirus 8 infection".)

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Literature review current through: Nov 2017. | This topic last updated: May 16, 2016.
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