Castleman Disease

Niiro Hiroaki

doi:10.14961/cra.31.75

Castleman disease（CD）is a very rare non-neoplastic lymphoproliferative disease with a characteristic pathological feature in lymph nodes, which was originally reported by Dr. Benjamin Castleman in 1956. The clinical course varies from a mild form to a very severe form with high mortality, suggesting a heterogenous pathogenesis of CD. CD is pathologically classified as hyaline vascular（HV）, plasma cell（PC）, and mixed type, while this disease is also clinically classified as unicentric（UCD）and multicentric（MCD）. The majority of patients with UCD exhibits HV type and can be cured by complete surgical resection of pathologic lymph nodes. On the other hand, the majority of patients with MCD exhibit PC type and complications in various organs along with constitutional symptoms, and thus often refractory to standard treatment. MCD with unknown etiology is designated as idiopathic MCD（iMCD）, and recently became specified as an incurable disease in Japan. This review describes the etiology and pathogenesis of CD, and focuses particularly on recent updates of the diagnosis and assessment of disease activity of iMCD. Steroids and cytotoxic agents had long been used for treatment of iMCD, however its prognosis was often aggravated by various complications such as infections and malignancies. Discovery of a pivotal role of IL-6 in the pathogenesis of iMCD has now made IL-6 blockade as the first-line treatment. Some patients with iMCD, however, are refractory to standard therapies including IL-6 blockade. A recent notable collaboration between CD experts nationally and internationally has helped further clarify the pathogenesis of this intractable disease, thus paving a new avenue for a novel therapeutic strategy in the future.

Castleman Disease

Bibliographic Information

Search this article

Description

Journal

Keywords

Details 詳細情報について

Export

Report a problem