Human Herpesvirus 8–Related Castleman Disease in the Absence of HIV Infection

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Background. Castleman disease (CD) in the context of human immunodeficiency virus (HIV) infection is well described. It is almost always multicentric (MCD) and linked to human herpesvirus 8 (HHV-8). There are limited published data surrounding HHV-8–related CD among HIV-negative patients.

Methods. From January 1995 through June 2012, we identified in a single center 18 HIV-seronegative patients with HHV-8–related CD. We report on their clinical, pathological, and laboratory features.

Results. All cases were multicentric. Patients were aged 42–83 years and were referred with a relapsing remitting syndrome of fever (94%), constitutional symptoms (100%), peripheral lymphadenopathy (100%), splenomegaly (72%), hepatomegaly (50%), and edema (28%). Kaposi sarcoma was observed in 9 cases. Anemia and serum markers of inflammation were present in all cases. Polymerase chain reaction for HHV-8 DNA was positive on blood samples in all cases, whereas only 12 of 16 patients tested had positive HHV-8 serology at diagnosis. All cases showed the classic histological features of MCD, and LANA-1 immunostaining identified HHV-8–infected plasmablasts in 16 of 16 tested cases. Reactive hemophagocytic syndrome (44%), autoimmune hemolytic anemia (33%), and lymphoma (22%) were the commonest associated complications. Remission was obtained with etoposide in 13 of 15 cases. Rituximab allowed prolonged remission off therapy in 10 cases. Death occurred in 3 patients not treated with rituximab. These features were similar to those described in HIV-positive HHV-8–related MCD. Comparison between these 18 cases and 12 HIV-negative HHV-8–unrelated MCD cases showed marked discrepancies.

Conclusions. HHV-8–associated MCD may be considered as a single clinicopathological entity regardless of HIV status.

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