Faculty Development Case of the Month: July 2021

Please view the July 2021 Case of the Month below:

Clinical Cases from Emory Division of Hospital Medicine Annual Clinical Vignette Conference: “Getting a Kick out of KICS”

Submitted by:
Patricia Hwang, MD
Assistant Professor of Medicine
Department of Medicine (Division of Hospital Medicine)
Emory University School of Medicine

Edited by Yelena Burklin, MD, FHM, FACP

STORY AND CASE

A 47-year-old male with a history of Acquired Immune Deficiency Syndrome (AIDS) marked by a CD4 count of 183, chronic kidney disease stage 3a with a baseline creatinine of 1.5 mg/dL, and a history of Castleman Disease (CD) that was previously treated with rituximab, presented initially with high fever, dyspnea, generalized weakness, and bilateral lower extremity swelling. Upon admission, patient remained persistently febrile with the oral temperature of 39.3 degrees Fahrenheit, he had tachycardia up to 120 BPM and a low to normotensive measured blood pressure.

His initial physical examination demonstrated shotty lymphadenopathy in the axilla and inguinal regions, marked bilateral lower extremity edema, as well as bibasilar crackles upon lung auscultation.

The initial laboratory results revealed a hemoglobin of 6.7 g/dL, platelet count of 67,000/µL, serum creatinine of 4.75 mg/dL, and blood urea nitrogen of 44 mg/dL.

He was started on intravenous antibiotics due to the presenting symptoms being attributed to sepsis, however there was no clinical improvement.

Blood and urine cultures remained sterile. Ventilation and perfusion scan was not characteristic of a pulmonary embolism and chest X-ray showed no acute abnormalities. As his acute renal failure persisted, patient required initiation of hemodialysis for fluid removal due to severe volume overload that was refractory to diuretic therapy.

The images and the reported results of the chest, abdominal, and pelvic computed tomography are provided below:

 

Computed Tomography (CT) of the chest, abdomen and pelvis revealed innumerable lymphadenopathy throughout.

Given low suspicion for an infectious process, patient was referred for an inpatient bone marrow biopsy.

What is your diagnosis?

Final diagnosis:

Bone marrow biopsy revealed a recurrence of Castleman Disease as well as findings suggestive of Kaposi Sarcoma Inflammatory Cytokine Syndrome (KICS).

Disease Trajectory of Interest:

Antibiotics were stopped and patient was started on rituximab. His symptoms, vital signs, and laboratory abnormalities began improving, which demonstrated favorable response to therapy.

DISCUSSION

KICS is a condition that has only been recently described in medical literature, and it is caused by excessive cytokine release that is associated with edema, cytopenia, and fever. KICS often mimics the appearance of sepsis but is unresponsive to antibiotics. Patients infected with Human Immunodeficiency Virus and Human Herpesvirus 8 (HHV-8) are predisposed to developing KICS. HHV-8 is also associated with CD. Our patient had known history of CD and presented with the signs and symptoms that only improved after immunomodulatory therapy was initiated.

Similar presentation of KICS may go unrecognized especially early in a patient’s presentation. KICS carries a mortality rate of up to 60%, and therefore, its timely recognition is required.

Our case highlights the importance of early referral for a bone marrow biopsy in patients with suggestive signs and symptoms who are within the high-risk patient population groups.

CITATIONS

• Karass M, Grossniklaus E, Seoud T, Jain S, Goldstein DA. Kaposi Sarcoma Inflammatory Cytokine Syndrome (KICS): A Rare but Potentially Treatable Condition. The Oncologist. 2017 May;22(5):623-625. doi: 10.1634/theoncologist.2016-0237. Epub 2017 Apr 19. PMID: 28424322; PMCID: PMC5423516.
• Cantos VD, Kalapila AG, Nguyen ML, Adamski M, Gunthel CJ, Experience with Kaposi Sarcoma Herpesvirus Inflammatory Cytokine Syndrome in a Large Urban HIV Clinic in the United States: Case Series and Literature Review, Open Forum Infectious Diseases, 2017; 4 (4):1-7 https://doi.org/10.1093/ofid/ofx196