Are patients with HHV-8 associated Castleman disease successfully treated with rituximab at risk of subsequently developing HHV-8 negative (idiopathic) Castleman disease? iMCD after HHV-8-associated MCD

• Published in: European journal of case reports in internal medicine 2023-06
• Main Authors: Ha, Gavin, Kwong, Kelsey, Tanaka, Bryce, Nishimura, Yoshito, Chong, Christina
• Format: Article
• Language: English
• ISSN: 2284-2594; 2284-2594
• DOI: 10.12890/2023_003904

Introduction: Multicentric Castleman disease (MCD) is a lymphoproliferative disorder characterized by lymph node histopathology and systemic symptoms. To our knowledge, there are no descriptions in the literature of long-term outcomes of human herpesvirus-8 (HHV-8)-associated MCD. Case Description: We report a case of a 70-year-old male living with human immunodeficiency virus and a history of human herpesvirus-8 (HHV-8)-associated MCD. The patient reported having had low-grade fever for two weeks. Extensive workup revealed systemic lymphadenopathy without evidence of autoimmune disease or malignancy. Lymph node biopsy was consistent with HHV-8-negative idiopathic MCD (iMCD). The patient was subsequently scheduled for anti-interleukin-6 therapy. Discussion: The present case is the first report of probable development of iMCD after long-term follow-up for HHV-8-associated MCD. The case illustrates the possible long-term consequences of MCD, suggesting the necessity of further research on the pathogenesis of CD. Conclusion: Given the uncertainty in the long-term outcomes of HHV-8-associated MCD, periodic surveillance of patients with a history of HHV-8-associated MCD is warranted. Prospective nationwide cohort studies comparing characteristics of HHV-8-associated MCD and iMCD would bring further insights.