Castleman's disease is not just a single disease but rather an uncommon, heterogeneous group of nonclonal lymphoproliferative disorders, which have a broad spectrum of clinical expression. Three histological types have been reported, along with several clinical forms according to clinical presentation, histological substrate and associated diseases. Interleukin-6, its receptor polymorphisms, the human immunodeficiency virus and the human herpes virus 8 are involved in the etiopathogenesis of Castleman's disease. The study of this disease has shed light on a syndrome whose incidence is unknown. Despite recent significant advances in our understanding of this disease and the increasing therapeutic experience with rituximab, tocilizumab and siltuximab, there are still difficult questions concerning its aetiology, prognosis and optimal treatment.
Keywords: Angiofollicular lymph node hyperplasia; Castleman's disease; Enfermedad de Castleman; Enfermedad de Castleman multicéntrica; Hiperplasia linfoide angiofolicular; Human herpes virus 8; Human immunodeficiency virus; Multicentric Castleman's disease; Rituximab; Siltuximab; Tocilizumab; Virus de la inmunodeficiencia humana; Virus del herpes humano tipo 8.
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