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Vol. 216. Issue 3.
Pages 146-156 (April 2016)
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Vol. 216. Issue 3.
Pages 146-156 (April 2016)
Review
Current diagnosis and treatment of Castleman's disease
Diagnóstico y tratamiento actual de la enfermedad de Castleman
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15
A. González García, M.Á. Moreno Cobo, J.L. Patier de la Peña
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Servicio de Medicina Interna, Hospital Universitario Ramón y Cajal, Madrid, Spain
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Table 1. Clinical, biological and evolutionary characteristics of Castleman's disease.
Abstract

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 etiology, prognosis and optimal treatment.

Keywords:
Castleman's disease
Multicentric Castleman's disease
Angiofollicular lymph node hyperplasia
Human immunodeficiency virus
Human herpes virus 8
Rituximab
Tocilizumab
Siltuximab
Resumen

La enfermedad de Castleman no es una única enfermedad. Bajo este epónimo se reúne un heterogéneo grupo de trastornos linfoproliferativos no clonales, muy infrecuentes, con un amplio espectro de expresión clínica. Se han descrito 3 tipos histológicos, junto con varias formas clínicas, según la forma de presentación, el sustrato histológico y las enfermedades asociadas. La interleucina 6, los polimorfismos del receptor de esta interleucina, el virus de la inmunodeficiencia humana y el virus herpes humano tipo 8 están implicados en la etiopatogenia y su estudio ha aportado luz al conocimiento de un síndrome cuya incidencia es desconocida. A pesar de avances recientes e importantes en su conocimiento y de la progresiva experiencia terapéutica con rituximab, tocilizumab y siltuximab, aún existen preguntas difíciles de contestar con los factores etiológicos, el abordaje terapéutico óptimo y el pronóstico.

Palabras clave:
Enfermedad de Castleman
Enfermedad de Castleman multicéntrica
Hiperplasia linfoide angiofolicular
Virus de la inmunodeficiencia humana
Virus del herpes humano tipo 8
Rituximab
Tocilizumab
Siltuximab

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