International Journal of Hematology

, Volume 90, Issue 3, pp 392–396 | Cite as

HHV8-positive, HIV-negative multicentric Castleman’s disease: early and sustained complete remission with rituximab therapy without reactivation of Kaposi sarcoma

  • Paolo Nicoli
  • Ubaldo Familiari
  • Marco Bosa
  • Tiziano Allice
  • Francesca Mete
  • Alessandro Morotti
  • Daniela Cilloni
  • Giuseppe Saglio
  • Angelo Guerrasio
Case Report


Multicentric Castleman’s disease (MCD) is a rare lymphoproliferative disorder with systemic symptoms and poor prognosis and is characterized by an abnormal proliferation of polyclonal plasmablasts in the mantle zone of B-cell follicles. The disease is found primarily in chronic HIV carriers and is usually strictly associated with human herpes virus type 8 (HHV-8) coinfection, which is believed to play a key role in the pathogenesis of MCD. The disease is also diagnosed in HIV-negative patients, who are usually elderly or immunosuppressed; however, in about half of these cases, no evidence of HHV8 infection is found. The anti-CD20 monoclonal antibody rituximab is now the preferred treatment for HIV-positive MCD. However, it is not clear whether rituximab is effective in HIV-negative patients with MCD, particularly in the HHV8-positive subset. We report here the clinical and biologic courses of two HIV-negative, HHV8-positive patients with MCD who were treated with rituximab. In both cases, a significant clinical improvement was observed after the first two infusions, which was shortly followed by a drop in HHV8 viremia to undetectable levels. Both patients underwent complete clinical remission, which persisted without relapse at 30 and 9 months of follow-up, respectively. No reactivation of the Kaposi sarcoma found in a lymph node of one of the patients was observed. Our report, along with additional data present in the literature, suggests that rituximab may be an appropriate and safe first-line therapy for HIV-negative, HHV8-positive MCD.


Lymphoproliferative disorder HHV8 virus Rituximab 


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Copyright information

© The Japanese Society of Hematology 2009

Authors and Affiliations

  • Paolo Nicoli
    • 1
  • Ubaldo Familiari
    • 2
  • Marco Bosa
    • 1
  • Tiziano Allice
    • 3
  • Francesca Mete
    • 1
  • Alessandro Morotti
    • 1
  • Daniela Cilloni
    • 1
  • Giuseppe Saglio
    • 1
  • Angelo Guerrasio
    • 1
  1. 1.Division of Hematology and Internal Medicine, Department of Clinical and Biological SciencesSan Luigi Gonzaga Hospital, University of TurinOrbassanoItaly
  2. 2.Pathology Department, Department of Clinical and Biological SciencesSan Luigi Gonzaga Hospital, University of TurinTurinItaly
  3. 3.Microbiology and Virology LaboratoryAmedeo di Savoia HospitalTurinItaly

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