Castleman Disease

Castleman’s disease (CD) was first described in 1954 and further defined in 1956 by Dr. Castleman (1) (Figure 13.1). He reported on patients possessing localized mediastinal lymph node enlargement that was characterized by redundancy of lymphoid follicles with germinal-center involution as well as marked capillary proliferation with endothelial hyperplasia in both follicular and interfollicular regions. Prior cases had been reported, but were anecdotal in nature (2, 3). In 1962, Lattes and Pachter evaluated 12 cases and suggested that these lymph nodes were hamartomatous in nature (4). Lee described refractory anemia associated with CD that responded to surgical resection 5, and by 1967, Tung and McCormack described 5 new cases and reviewed the 62 cases described to that point in the literature (6), highlighting the potential for associated hypochromic anemia, hypergammaglobulinemia, and bone marrow plasmacytosis.