Abstract

Multicentric Castleman disease (MCD) is a lymphoproliferative disorder caused by human herpesvirus 8 (HHV8) infection HIV associated MCD (HIV-MCD) presents with various clinical symptoms. Many HIV-negative MCD patients are often treated with anti-human interleukin-6 (IL6) receptor monoclonal antibodies (tocilizumab), and successful results have been reported. IL-6 plays an important role in the development of both HIV-positive and HIV-negative MCD; however, the efficacy of tocilizumab in HIV-MCD patients is unknown. We herein report the clinical and biologic courses of two HIV-MCD patients treated with tocilizumab. In both cases, a significant and rapid clinical improvement was observed after the first infusion. However, the treatment efficacy was not maintained for a long period, and relapse occurred at 15 and 22 weeks, respectively. Both patients received rituximab and subsequently achieved complete clinical remission. Our report, in addition to data presented in the literature, suggests that tocilizumab could be an initial treatment option in patients with HIV-MCD.

Highlights

  • Multicentric Castleman disease (MCD) is a lymphoproliferative disorde, and HIV-associated MCD (HIV-MCD) is caused by human herpesvirus 8 (HHV8) infection in HIV-positive patients [1]

  • HIV-negative MCD patients treated with tocilizumab exhibit very higher levels of human IL6 (hIL6) after treatment because hIL6 receptors are blocked by IL-6 receptor antibodies [2]

  • This suggests that HHV8 replication due to immunodeficiency was the primary pathogenesis of HIV-MCD in these cases, resulting in a vicious cycle of hIL6 production being blocked by the inhibition of IL6 signal transmission following the administration of tocilizumab

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Summary

Introduction

Multicentric Castleman disease (MCD) is a lymphoproliferative disorde, and HIV-associated MCD (HIV-MCD) is caused by human herpesvirus 8 (HHV8) infection in HIV-positive patients [1]. HIV-MCD presents with various clinical symptoms, including fever, swelling of the spleen, liver and systemic lymph nodes and abnormalities in laboratory values, such as findings of anemia, thrombocytopenia or hypergamma-globulinemia, as well as a low albumin, or high C-reactive protein (CRP) level. We report the results of two HIV-MCD patients treated with tocilizumab.

Results
Conclusion
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