Treatment of multicentric castleman’s disease: Research advances / 国际药学研究杂志

ABSTRACT

Castleman’s disease (CD) is a rare lymphoproliferative disorder which comprises two distinct clinical subtypes unicentric CD (UCD) and multicentric CD (MCD). Unlike UCD, which is a curable disease with surgical resection, MCD is a disease with systemic symptoms and high mortality rate (the 5-year mortality rate could be as high as 35%). There is no standard of care for MCD. Current treatment options include observation, glucocorticoids, chemotherapy (with or without rituximab) and immunomodulatory agents. Progress have been made during the recent years, interleukin-6 (IL-6) and human herpes virus 8 (HHV-8) have been recognized as key factors involved in the pathogenesis of MCD. Accordingly, drugs targeting these factors (especially IL-6) have been developed to treat MCD. In this paper, we try to review the current treatment options and new emerging therapies for MCD which might help Chinese clinicians to learn more about this rare disease.