Post-transplant Kaposi Sarcoma with Kaposi Sarcoma inflammatory cytokine syndrome: A case report with literature review

• Post-transplant KS can present aggressively with unusual hepatic involvement and acute liver failure • KICS is a rare but highly fatal form of the disease suspected when there is high HHV8 viral load with persistent inflammation, multi-organ dysfunction, elevated IL-6/IL-10 • Overlapping infectious, malignant, and inflammatory processes create diagnostic challenges • Multi-system complications eliminate most treatment options - infection, organ failure, and cytopenias create therapeutic dead ends • Early mTOR inhibitor switch (CNI to sirolimus) beneficial in post-transplant KS • There is a vital need for: Earlier recognition of disease. Novel therapeutics viable despite multi-system involvement Kaposi Sarcoma (KS) is a vascular malignancy driven by Human Herpes Virus 8 (HHV-8), commonly occurring in immunocompromised states. Hepatic involvement and development of Kaposi Sarcoma Herpesvirus Inflammatory Cytokine Syndrome (KICS) in post-liver transplant recipients is rare and hence not well described. A 59-year-old Caucasian woman developed cholestatic liver dysfunction six months following orthotopic liver transplantation for primary biliary cirrhosis. Initial imaging revealed a hilar and peribiliary soft tissue abnormality, with fine needle aspiration confirming Kaposi Sarcoma. Her clinical course was complicated by multidrug-resistant gram-negative septic encephalopathy, progressive hepatic failure, and acute kidney injury requiring continuous renal replacement therapy. High HHV-8 viral load and persistent systemic inflammation despite appropriate antimicrobial therapy raised suspicion for KICS. Despite immunosuppressive dose reduction and transition to sirolimus, she developed with multi-organ failure, precluding standard chemotherapy with pegylated liposomal doxorubicin. This case highlights the aggressive nature of post-transplant hepatic KS and its potential evolution into KICS. The unique presentation of hilar involvement causing obstructive hepatopathy creates diagnostic challenges and severely limits therapeutic options. The development of refractory sepsis and multi-organ dysfunction associated with KICS necessitates novel treatment approaches beyond conventional immunosuppression reduction. Clinicians should maintain high suspicion for KS and KICS in post-transplant patients presenting with unexplained systemic inflammation and hepatic dysfunction. Early recognition and development of alternative therapeutic strategies are crucial for improving outcomes in this challenging clinical scenario.