• Medicina clinica · Mar 2024

    Review

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

    • Roberto Pertusa Mataix, Daniel Loaiza Cabello, and José Salvador García Morillo.
    • Servicio de Medicina Interna, Departamento de Enfermedades Autoinmunes, Hospital Universitario Virgen del Rocío, Sevilla, España. Electronic address: roberpm97@gmail.com.
    • Med Clin (Barc). 2024 Mar 22; 162 (6): 283290283-290.

    AbstractCastleman'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.Copyright © 2023 Elsevier España, S.L.U. All rights reserved.

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