Experience with Kaposi Sarcoma Herpesvirus Inflammatory Cytokine Syndrome in a Large Urban HIV Clinic in the United States: Case Series and Literature Review

Abstract

In 2010, a new entity, characterized by the classical signs and symptoms of Kaposi sarcoma herpesvirus–associated multicentric Castleman’s disease (KSHV-MCD) in the absence of pathologic evidence of MCD, was described in individuals living with HIV. This syndrome was named KSHV inflammatory cytokine syndrome (KICS). It carries mortality rates of up to 60%. To date, there are no standard therapies. Treatment regimens studied in clinical trials for MCD disease are used in cases of KICS.

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

“…KICS is uncommon with less than 20 cases confirmed so far. It can be easily misdiagnosed in immunocompromised patients with HIV/AIDS; differential diagnoses include septic shock, KSHV-MCD, PEL, and KS-related immune reconstitution inflammatory syndrome (KS-IRIS) [2,9]. Comprehensive workups failed to find any other infection source in our patient.…”

“…We presented a case consistent with the newly recognized KICS in a HIV/HHV8 co-infected patient. The working diagnostic criteria for KICS have been developed by Polizzotto's group [2,4,5]. KICS is defined with minimal lymphadenopathy/splenomegaly and negative pathologic nodal changes in the setting of lower CD4 count (<100 cells/μl).…”

“…KSHV viremia during symptoms remains high, whereas HIV viremia is typically low in KSHV-MCD [12]. Finally, KICS is also distinguished from KSHV-MCD by their CD4 counts, where CD4 cells in KICS are typically much lower (<100 cells/μl) than in KSHV-MCD (>200 cells/μl) [2].…”

“…Although our patient did develop bilateral pleural effusions, PEL was excluded by negative cytology of pleural fluid. On the other hand, KS-IRIS presents as acute deterioration of KS involvement of new systems (gastrointestinal, pulmonary, lymphatic) after starting ART [2,15]. A temporal association within 14 weeks of ART initiation usually exists, while KSHV and HIV viremia remains low accompanied by >50 cells/μl CD4 count increases from pre-ART levels [2,16].…”

“…On the other hand, KS-IRIS presents as acute deterioration of KS involvement of new systems (gastrointestinal, pulmonary, lymphatic) after starting ART [2,15]. A temporal association within 14 weeks of ART initiation usually exists, while KSHV and HIV viremia remains low accompanied by >50 cells/μl CD4 count increases from pre-ART levels [2,16]. KS-IRIS is unlikely in this patient as he had been on ART for more than one year with substantial KSHV viremia in the setting of very low CD4 cell counts during his entire hospitalization.…”