Complete remission in 4 patients with human herpesvirus 8-associated multicentric Castleman disease using rituximab and liposomal doxorubicin, a novel chemotherapy combination.

Plasmablastic-type multicentric Castleman disease (PMCD) is a rare, aggressive lymphoproliferative disorder first described by Frizzera and colleagues in 1983.1 Today, the disease is most commonly diagnosed in individuals infected with human immunodeficiency virus (HIV) type 1. It has been shown that PMCD is associated with Kaposi sarcoma, and a majority of patients with both conditions are infected by human herpesvirus 8 (HHV8), also known as Kaposi sarcoma–associated herpesvirus.2-4 The diagnosis is based on clinical, pathologic, and laboratory findings. The common clinical course is characterized by recurrent attacks of fever, generalized lymphadenopathy, fatigue, splenomegaly, hepatomegaly, and anemia. The pathologic examination of involved lymph nodes or spleen frequently shows an increased number of follicles, some of which have atrophic, hyalinized germinal centers, increased vascularity, and sheets of plasma cells within the interfollicular areas. A variable number of so-called plasmablasts, from scattered perifollicular cells to overt lymphoma, can be identified by immunohistochemical staining (IHC) with HHV-8. We have recently showed that in a significant proportion of these cases, the same cells are coinfected with Epstein-Barr virus (EBV).5 Coexistent Kaposi sarcoma is often present in lymph nodes involved by PMCD or elsewhere. The initial disease can be self-limited; however, the prognosis is usually poor and most patients die with active PMCD or progress to HHV-8–associated non-Hodgkin lymphoma (NHL) within months.6