Purpose of review: In this review we will discuss the recent findings in HIV-associated multicentric Castleman disease. On the basis of current knowledge on pathophysiology, we will illustrate different therapeutic approaches and try to provide guidelines at least for the initial care of the disease.
Recent findings: On the basis of pathological and virological data, pathophysiology appears to conjugate both proliferation of human herpesvirus (HHV-8) infected plasmablasts and replication of HHV-8. Therefore, recent therapies have targeted the infected cells using chemotherapy or rituximab, an anti-CD20 monoclonal antibody or both, and the virus replication by using valganciclovir, a potent antiviral drug usually used against cytomegalovirus.
Summary: Etoposide is the most effective first-line therapy for active multicentric Castleman disease. Rituximab can be used after an initial control of the attack and provides a 1-yearremission rate above 70%. Exacerbation of Kaposi sarcoma lesions, observed in half of the patients with previous Kaposi sarcoma lesions, may represent a limitation.Valganciclovir effectively suppresses HHV-8 replication both in vitro and in vivo, and reduction in HHV-8 viremia associated with clinical improvement has been suggested in short series of patients with multicentric Castleman disease treated with valganciclovir.