A Rare Case of the Newly Recognized Kaposi Sarcoma Herpesvirus-Associated Disease

Qunfang Li; Faria Ali; Vivek Kak; Richard Santos

doi:10.7759/cureus.8784

A Rare Case of the Newly Recognized Kaposi Sarcoma Herpesvirus-Associated Disease

Cureus. 2020 Jun 23;12(6):e8784. doi: 10.7759/cureus.8784.

Authors

Qunfang Li¹, Faria Ali², Vivek Kak³, Richard Santos²

Affiliations

¹ Hematology and Oncology, Henry Ford Health System, Detroit, USA.
² Internal Medicine, Henry Ford Allegiance Health, Jackson, USA.
³ Infectious Disease, Henry Ford Allegiance Health, Jackson, USA.

Abstract

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.

Keywords: hiv; il-10; interleukin (il)-6; kshv inflammatory cytokine syndrome (kics); multicentric castleman disease (mcd).

Publication types

Case Reports