Faria Ali Qunfang Li Akhil Rahman Vivek Kak Michael Somero Chidamber Alamelumangapuram Richard Santos
Henry Ford Health System
Introduction: Kaposi sarcoma herpesvirus (KSHV) is associated with Kaposi sarcoma, primary effusion lymphoma and multicentric Castleman disease (KSHV-MCD) in patients infected with human immunodeficie..
Introduction: Kaposi sarcoma herpesvirus (KSHV) is associated with Kaposi sarcoma, 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. Case Report: A 33-year-old African American male with a prior history of syphilis, HIV/AIDS on Triumeq and stage IV Kaposi sarcoma on Doxorubicin, presented with worsening fatigue, nausea, vomiting, myalgias, dyspnea and anasarca. His CD4 count remained at 33 cells/ul despite a low HIV-1 viral load. He was febrile and treated with multiple antibiotics, while extensive workups with cultures ruled out infectious causes. He had persistent hyponatremia, hypoalbuminemia, and then developed anemia and thrombocytopenia. Biopsy of his lymph node excluded KSHV-MCD. His significantly increased C-reactive protein and the absence of lymphadenopathy/splenomegaly on CT images led to the suspicion of KICS. KICS was further confirmed by high KSHV PCR (14855 copies/ml), massive elevation of cytokines IL-6 and IL-10. Even with aggressive immunoglobulin and supportive treatments, he died of multi-organ failure one month later. Discussion: Although both disorders exhibit signs of substantial inflammation, KICS is a different entity from KSHV-MCD. KICS is defined with no lymphadenopathy/splenomegaly and negative pathologic nodal changes in the setting of low CD4 count (/ul). Standard therapy is still under investigation due to its rarity and high mortality, whereas combination of Rituximab and Doxorubicin may lead to clinical remission. Early diagnosis and treatment initiation are crucial to improve survival of this under-recognized KSHV-associated disease.