Does Kaposi's sarcoma predict multicentric Castleman disease in the presence of generalized lymphadenopathy?

Abstract

Multisystemic Castleman disease (MCD) can be associated with HHV8 infection, which involves the mantle zone of follicles. This condition results in an increase number of HHV8-positive (HHV8+) plasmablasts that multiply and amalgamate to form plasmablastic B-cell lymphoma. All previously reported cases of HIV-positive patients with MCD were coinfected with HHV8. Twelve cases of HIV- MCD that are HHV8+ were encountered in the literature, three of them developed lymphoma, and none of those cases were reported to have Kaposi's sarcoma (KS). We report a unique case of HIV-negative elderly woman with preexisting KS that presented to the hospital with recurring nausea, vomiting, and fever, assessment revealed diffuse lymphadenopathy. Axillary lymph node biopsy showed HHV8+ MCD with foci of microlymphoma. Despite the treatment with high-dose steroids, she developed multisystem failure that lead to her death.