[Castleman's disease, pathophysiology, advances in diagnosis and treatment].

Autor: Pertusa Mataix R; Servicio de Medicina Interna, Departamento de Enfermedades Autoinmunes, Hospital Universitario Virgen del Rocío, Sevilla, España. Electronic address: roberpm97@gmail.com., Loaiza Cabello D; Servicio de Medicina Interna, Departamento de Enfermedades Autoinmunes, Hospital Universitario Virgen del Rocío, Sevilla, España., García Morillo JS; Servicio de Medicina Interna, Departamento de Enfermedades Autoinmunes, Hospital Universitario Virgen del Rocío, Sevilla, España.
Jazyk: English; Spanish; Castilian
Zdroj: Medicina clinica [Med Clin (Barc)] 2024 Mar 22; Vol. 162 (6), pp. 283-290. Date of Electronic Publication: 2023 Nov 27.
DOI: 10.1016/j.medcli.2023.10.013
Abstrakt: Castleman's disease (CD) encompasses a heterogeneous set of reactive lymphoproliferative processes that share well-defined histologic features. CD is considered a rare or minority disease. The incidence of CD is not fully known, although it is estimated at less than 1 per 100,000 inhabitants. It has a bimodal distribution (30-40 years and then 60-80 years). The incidence is similar in both sexes, although the unicentric variant seems to have a slight predominance in women with a 2:1 ratio. CD is classified into a hyalinovascular form (this being the most frequent) and a plasmocellular form, related to the HIV and VHH-8 viruses, which together with other autoimmune mechanisms develop hyperproduction of interleukin-6 (IL-6) by B lymphocytes. There are different lines of treatment, where the use of anti IL-6 stands out, being siltuximab the most used as orphan drug in this pathology.
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Databáze: MEDLINE