**Multicentric Castleman's Disease**

Moosa Patel, Vinitha Philip, Atul Lakha, Sugeshnee Pather, Muhammed Faadil Waja, Lucille Singh and Mohamed Arbee

Additional information is available at the end of the chapter

http://dx.doi.org/10.5772/61709

#### **Abstract**

Castleman's disease (CD) is a lymphoproliferative disorder, manifesting clinically as uni‐ centric or multicentric disease and pathologically as hyaline vascular, plasma cell or mixed variants.

Multicentric Castleman's disease (MCD) is the most common form of CD encountered at Chris Hani Baragwanath Academic Hospital (CHBAH). From being a rare disease, MCD has increased in the last five years, primarily as a result of the association of human im‐ munodeficiency virus (HIV), being highly prevalent in our patient population. The domi‐ nant clinical manifestations of the disease include constitutional symptoms, fever, anaemia, lymphadenopathy and hepatosplenomegaly.

We present a series of 35 adult patients with MCD, who were seen over a 25-year period at CHBAH, and highlight the similarities and differences compared with other published series.

Based on our local experience, we observed that the prognosis of HIV-associated MCD has improved with optimization and control of HIV replication (use of combination anti‐ retroviral therapy), prophylaxis and treatment of opportunistic infections, as well as eto‐ poside and rituximab based chemotherapy.

In the setting of HIV, MCD should no longer be regarded as a rare disease with a fatal outcome.

**Keywords:** Multicentric Castleman's disease, Human immunodeficiency virus infection, Human herpes virus-8, South Africa, immunosuppression

#### **1. Introduction**

Castleman's disease (CD), also known as angiofollicular or giant lymph node hyperplasia, is a rare B-cell lymphoproliferative disorder, first described by Benjamin Castleman in a series

© 2015 The Author(s). Licensee InTech. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

of patients in 1956 [1]. It is a heterogeneous disorder, manifesting clinically as a unicentric (solitary; localised) or multicentric disease and pathologically as hyaline vascular, plasma cell or mixed variants [2–6].

The multicentric variety is aetiologically linked to human herpes virus-8 (HHV-8) [7]. It is strongly associated with immunosuppression and is now being encountered with increasing frequency in patients with HIV (human immunodeficiency virus) infection [8].

This review will focus on CD, with particular reference to Multicentric Castleman's disease (MCD), and it will include a description of the disease as seen at Chris Hani Baragwanath Academic Hospital (CHBAH), Johannesburg, South Africa over a 25-year period (1990 to 2014). The renewed interest in MCD stems from its association with HIV, particularly in areas such as sub-Saharan Africa where HIV has reached pandemic proportions, with South Africa being home to approximately 6.4 million people living with HIV/AIDS (acquired immunodeficiency syndrome) [9].
