Case Presentation: Mr. J is a 47 year old male with a history of Acquired Immune Deficiency Syndrome (CD4 count of 183), chronic kidney disease stage 3 (baseline creatinine of 1.5 mg/dL) and Castleman Disease (CD, previously treated with rituximab) who presented initially with complaints of fevers, dyspnea, generalized weakness, and swelling to the lower extremities. He was found to have persistent fevers of up to 39.3 degrees Fahrenheit as well as tachycardia to the 120s with low to normotensive blood pressures. His initial physical exam demonstrated shotty lymphadenopathy in his axilla and inguinal regions, marked edema of his lower extremities as well as crackles in the bilateral lower lobes of his lungs.His initial laboratory results showed several derangements. He had acute anemia with a hemoglobin of 6.7 g/dL, thrombocytopenia with a platelet count of 67,000/mcL, acute kidney injury with a creatinine of 4.75 mg/dL and blood urea nitrogen of 44 mg/dL. His symptoms were initially thought to be due to sepsis and he was started on broad spectrum antibiotics. Nephrology, infectious diseases and hematology/oncology were consulted to assist with management.
Mr. J failed to improve on antibiotics, however, and his initial presenting symptoms persisted. Workup including blood and urine cultures demonstrated no growth, ventilation and perfusion scan was not consistent with a pulmonary embolism and chest X-ray showed no acute abnormalities. He also required dialysis for fluid removal due to severe volume overload that was refractory to diuretics.
CT chest, abdomen and pelvis showed innumerable lymphadenopathy throughout. Antibiotics were stopped as his symptoms were thought to be most likely due to hematologic/oncologic issues. He eventually underwent a bone marrow biopsy which showed a recurrence of his CD as well as findings suggestive of Kaposi Sarcoma Inflammatory Cytokine Syndrome (KICS). He was started on rituximab and his symptoms, laboratory abnormalities and vital signs began to improve.
Discussion: KICS is a disease that has only been recently described in medical literature and is a condition caused by excessive cytokine release that is associated with edema, cytopenias, and fevers. It often mimics the appearance of sepsis but is unresponsive to antibiotics. It is a disease that affects those infected with Human Immunodeficiency Virus and human herpesvirus 8 (HHV-8). HHV-8 is also associated with CD. Mr. J, with his known history of CD presented with these exact signs and symptoms which only improve after immunomodulatory therapy was started.
Conclusions: Given its relatively new description and similar presentation to sepsis, KICS may go unrecognized especially early in a patient’s presentation. It is important to learn more about this new syndrome and consider it, especially in high risk patient populations.