Case Report: Synchronous Manifestations of Kaposi Sarcoma Herpesvirus-Associated Disorders.

BACKGROUND

Kaposi sarcoma herpes virus (KSHV) is associated with multiple clinical manifestations, including primary effusion lymphoma, an aggressive CD38+ B cell lymphoma with a plasmablastic phenotype.

CASE: This case describes an antiretroviral therapy-adherent person with HIV who presented with concurrent KSHV-related disorders of Kaposi sarcoma (KS), multicentric Castleman disease (MCD), and extracavitary primary effusion lymphoma (EC-PEL). Single-cell RNAseq (scRNAseq) and multiplex immunohistochemistry (mIHC) provide detailed insights into differences in cellular composition and viral and cellular transcriptomic differences between these diseases. Moreover, the case describes a long-term remission with single-agent anti-CD38 antibody, daratumumab, in a chemotherapy-refractory case.

CONCLUSION

This case highlights the differing clinical manifestations of KSHV and the efficacy of immunotherapy in KSHV-associated primary effusion lymphoma.