The many facets of Kaposi sarcoma-associated herpesvirus/human herpes virus-8 multicentric Castleman disease: clinical spectrum, diagnostic innovations, and therapeutic management
David Boutboul, Romain Stammler, Yannick DieudonnéBackground
Castleman diseases encompass three distinct entities: unicentric Castleman disease, KSHV/HHV-8-associated multicentric Castleman disease (MCD), and idiopathic MCD. HHV-8 MCD is a life-threatening B-cell lymphoproliferative disorder driven by KSHV/HHV-8 infection of B cells, resulting in systemic inflammation and diverse clinical phenotypes.
Objective
To summarize current knowledge of HHV-8 MCD pathogenesis, clinical presentations, autoimmune complications, and diagnostic advances.
Methods
This review synthesizes existing literature on HHV-8 MCD, with particular attention to emerging diagnostic techniques, including liquid-phase detection of circulating viroblasts.
Results
Treatment approaches center on rituximab-based regimens, with outcomes varying between HIV-positive and HIV-negative populations. Novel diagnostic tools combining flow cytometry and viral transcript detection offer rapid identification of disease flares.
Conclusion
These emerging diagnostic approaches may improve management of HHV-8 MCD, particularly in emergency settings where rapid flare identification is critical.