Title:An HHV-8 Positive HIV Negative Multicentric Castleman’s Disease, who Responded well to Rituximab Alone
Volume: 20
Issue: 1
Author(s): Dimitris Kounatidis, Dimitra Rontogianni, Dimitrios Sampaziotis, Maria Vardaka, Chara Giatra, Christodoulos Dolapsakis, Evangelia Margellou and Natalia G. Vallianou*
Affiliation:
- Department of Internal Medicine, Evangelismos General Hospital, Athens,Greece
Keywords:
Multicentric castleman disease, HHV-8 positive, HIV negative, rituximab, lymphoproliferative, cytokine.
Abstract:
Background: Multicentric Castleman Disease (MCD) presents with enlarged lymph
nodes in multiple regions and systemic inflammatory symptoms, due to the dysregulation of cytokines,
most commonly interleukin-6 (IL-6). Human herpes virus-8 (HHV-8) is strongly related to
MCD (HHV-8-associated MCD) and is being implicated in cytokine dysregulation in patients, the
majority of whom are HIV positive or immunosuppressed. Preferred treatment of HHV-8-
associated MCD depends on the presence or not of concurrent Kaposi sarcoma and on whether the
patient has life-threatening organ failure or poor performance status thought to be related to HHV-
8-associated MCD.
Case Presentation: Herein, we describe a female patient with HHV-8 positive, HIV negative MCD,
who responded well to the administration of rituximab once weekly for four weeks alone for three
cycles.
Conclusion: HHV-8 positive, HIV negative MCD treatment modalities are only anecdotal due to
the rarity of this form of MCD. Administration of rituximab alone seems to be beneficial among
patients with good performance status and the absence of life-threatening organ failure in cases of
HHV-8 positive, HIV negative MCD.
