The Kaposi sarcoma (KS) herpesvirus (KSHV), also known as human herpesvirus 8, is the causative agent of KS but is also pathogenetically linked to several lymphoproliferative disorders, including primary effusion lymphoma (PEL)/intracavitary (EC) PEL, KSHV-associated multicentric Castleman disease (MCD), KSHV+ diffuse large B-cell lymphoma, and germinotropic The many KSHV-associated disorders could co-occur and share symptoms. KSHV, like Epstein-Barr virus (EBV), is a lymphotropic gammaherpesvirus that is particularly found in immune-compromised people’s aberrant lymphoid proliferations. Notably, both KSHV and EBV have the ability to infect and convert the same B cell, as demonstrated in KSHV+ EBV+ PEL/EC-PEL.

The processes through which KSHV causes lymphoproliferative diseases were assumed to be linked to the expression of a few transforming viral genes that can influence cellular proliferation and survival. There were significant differences between KSHV-MCD and PEL/EC-PEL, the two most common KSHV-associated lymphoid proliferation, in terms of viral associations, viral gene expression patterns, and cellular differentiation stage as reflected by the phenotype and genotype of the infected abnormal B cells. Treatment advances had improved results, but death rates remained high. The growing understanding of KSHV biology, clinical aspects of KSHV-related disorders, and emerging clinical therapies should lead to more effective and focused therapeutic approaches.