Castleman’s Disease

Castleman’s disease (CD) is a rare and relatively unknown lymphoproliferative disorder, with benign hyperplastic lymph nodes. The disease was first reported in 1956 by Benjamin Castleman, a pathologist from the Massachusetts General Hospital (Castleman and Towne, N Engl J Med 250:1001–1005, 1954). In this first case report, Castleman described a 60-year-old male with a mediastinal mass. Histology showed lymph node hyperplasia and follicles with small, hyalinized foci. Subsequently, in 1956, he described a series of 13 cases of localized asymptomatic mediastinal masses based on lymph node hyperplasia on X-ray (Castleman et al., Cancer 9:822–830, 1956). All of the patients described in these early papers had localized disease, which is now termed unicentric Castleman’s disease (UCD). In contrast, multicentric Castleman’s disease (MCD) is a systemic disease with generalized peripheral lymphadenopathy, hepatosplenomegaly, frequent episodes of fever and night sweats. In this chapter, we will discuss the pathophysiology, epidemiology, clinical presentation, diagnostic procedures, treatment and prognosis of both forms of CD.