Kaposi sarcoma herpesvirus (KSHV) inflammatory cytokine syndrome (KICS): a case study

Abstract

Background: Kaposi sarcoma herpesvirus-inflammatory cytokine syndrome (KICS) is a rare, life-threatening condition associated with Kaposi sarcoma and systemic immune dysregulation induced by Human Herpesvirus 8 (HHV-8). With a mortality rate approaching 60%¹ (Goncalves, Ziegelbauer, Uldrick, Yarchoan in Curr Opin HIV AIDS 12(1):47-56, 2017), KICS poses significant diagnostic and therapeutic challenges. This report examines a case of KICS in a patient with well-controlled HIV, emphasising the clinical complexities, treatment strategies, and the need for heightened awareness.

Case presentation: A 59-year-old British male with a controlled HIV infection on Bictegravir/Emtricitabine/Tenofovir alafenamide presented with fever, malaise, lymphadenopathy, splenomegaly, and a purplish plantar plaque. Laboratory findings included anaemia, thrombocytopenia, hypoalbuminemia, hyponatremia, elevated inflammatory markers, and a high HHV-8 level. Diagnosis of HHV-8 positive lymph nodes and Kaposi sarcoma on the plantar aspect was confirmed. The patient was treated with Foscarnet, steroid, Rituximab, Tocilizumab, intravenous immunoglobulin (IVIG), and Paclitaxel, reducing viral load and improving cell count. This case highlights the complexities of managing Kaposi sarcoma within the realm of immune complex syndrome.

Conclusions: Our case report underscores the critical need for heightened awareness and recognition of KICS, given its rarity and unique clinical characteristics. By elucidating the complex interrelationships between Kaposi sarcoma, inflammatory cytokines, and immune dysregulation, we aim to contribute to the existing knowledge base and facilitate improved diagnosis, management, and therapeutic interventions for this challenging syndrome. Further research is warranted to explore novel treatment modalities and unravel the underlying mechanisms driving KICS.

Keywords: Human herpesvirus 8 (HHV-8); Human immunodeficiency virus (HIV); Kaposi sarcoma herpesvirus (KSHV); Kaposi sarcoma herpesvirus- inflammatory cytokine syndrome (KICS).

Fig. 1

Right plantar rash of the patient

Fig. 2

CD4 count trend of the patient

Fig. 3

PET-CT findings of the patient

Fig. 4

PET-CT findings of the patient

Fig. 5

A micrograph (100x magnification) showing the presence of irregularly shaped ectatic vascular channels which dissect into dermal collagen (black arrow). Within some of these neoplastic vascular channels are entrapped native vessels (red arrow), a histologic feature known as promontory sign and is classic to Kaposi sarcoma

Fig. 6

A higher power (200x magnification) of these abnormal vascular channels (black arrow) and the promontory sign (red arrow)

Fig. 7

HHV-8 immunostain highlights the endothelial cells lining the neoplastic vascular channels

Fig. 8

HHV-8 qPCR monitoring

Fig. 9

Cytokine profile (IL-10 & IL 18) before and after treatment