Are patients with HHV-8 associated Castleman disease successfully treated with rituximab at risk of subsequently developing HHV-8 negative (idiopathic) Castleman disease?
  • Gavin Ha
    Department of Medicine, John A. Burns School of Medicine, University of Hawai'i, Honolulu, HI, USA
  • Kelsey Kwong
    Department of Medicine, John A. Burns School of Medicine, University of Hawai'i, Honolulu, HI, USA
  • Bryce Tanaka
    Department of Medicine, John A. Burns School of Medicine, University of Hawai'i, Honolulu, HI, USA
  • Yoshito Nishimura
    Department of Medicine, John A. Burns School of Medicine, University of Hawai'i, Honolulu, HI, USA
  • Christina Chong
    Department of Medicine, John A. Burns School of Medicine, University of Hawai'i, Honolulu, HI, USA

Keywords

multicentric Castleman disease, human immunodeficiency virus, interleukin 6, siltuximab

Abstract

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.

VIEW THE ENTIRE ARTICLE

References

  • Fajgenbaum DC, Uldrick TS, Bagg A, Frank D, Wu D, Srkalovic G et al. International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. Blood 2017;129:1646-57.
  • Nishikori A, Nishimura MF, Nishimura Y, Otsuka F, Maehama K, Ohsawa K et al. Idiopathic Plasmacytic Lymphadenopathy Forms an Independent Subtype of Idiopathic Multicentric Castleman Disease. Int J Mol Sci 2022;23:10301.
  • Nishimura Y, Fajgenbaum DC, Pierson SK, Iwaki N, Nishikori A, Kawano M et al. Validated international definition of the thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly clinical subtype (TAFRO) of idiopathic multicentric Castleman disease. Am J Hematol 2021;96:1241-1252.
  • Nishimura MF, Nishimura Y, Nishikori A, Yoshino T, Sato Y. Historical and pathological overview of Castleman disease. J Clin Exp Hematop 2022;62:60-72.
  • Pria AD, Pinato D, Roe J, Naresh K, Nelson M, Bower M. Relapse of HHV8-positive multicentric Castleman disease following rituximab-based therapy in HIV-positive patients. Blood 2017;129:2143-147.
  • Ren N, Ding L, Jia E, Xue J. Recurrence in unicentric castleman's disease postoperatively: a case report and literature review. BMC Surg 2018;18:1.

    • Views: 271
    HTML downloads: 67
    PDF downloads: 293

    Published: 2023-06-20
    Issue: 2023: Vol 10 No 7 (view)

    How to cite:
    1.
    Ha G, Kwong K, Tanaka B, Nishimura Y, Chong C. 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. EJCRIM 2023;10 doi:10.12890/2023_003904.

    Most read articles by the same author(s)