Kaposi sarcoma herpesvirus (KSHV) is associated with Kaposi sarcoma (KS), primary effusion lymphoma, and multicentric Castleman disease (KSHV-MCD) in patients infected with human immunodeficiency virus (HIV). We present a case consistent with a newly recognized KSHV inflammatory cytokine syndrome (KICS), distinct from KSHV-MCD. Although both disorders exhibit signs of substantial inflammation, KICS has minimal lymphadenopathy/splenomegaly and negative pathologic nodal changes in the setting of low CD4 count. KICS is easily misdiagnosed as severe sepsis or other KS-related diseases in HIV/AIDS patients and carries a high mortality. Standard therapy is still under investigation due to its rarity, whereas the treatment regimen for KSHV-MCD may lead to clinical remission. Early recognition and prompt management are crucial to improve the survival of the under-recognized KICS.
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| http://dx.doi.org/10.7759/cureus.8784 | DOI Listing |
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