HHV8-positive, HIV-negative multicentric Castleman's disease: early and sustained complete remission with rituximab therapy without reactivation of Kaposi sarcoma.
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.
Nicoli
Paolo
P
Division of Hematology and Internal Medicine, Department of Clinical and Biological Sciences, San Luigi Gonzaga Hospital, University of Turin, Regione Gonzole 10, 10043, Orbassano, Turin, Italy.
Familiari
Ubaldo
U
Pathology Department, Department of Clinical and Biological Sciences, San Luigi Gonzaga Hospital, University of Turin, Turin, Italy.
Bosa
Marco
M
Division of Hematology and Internal Medicine, Department of Clinical and Biological Sciences, San Luigi Gonzaga Hospital, University of Turin, Regione Gonzole 10, 10043, Orbassano, Turin, Italy.
Allice
Tiziano
T
Microbiology and Virology Laboratory, Amedeo di Savoia Hospital, Turin, Italy.
Mete
Francesca
F
Division of Hematology and Internal Medicine, Department of Clinical and Biological Sciences, San Luigi Gonzaga Hospital, University of Turin, Regione Gonzole 10, 10043, Orbassano, Turin, Italy.
Morotti
Alessandro
A
Division of Hematology and Internal Medicine, Department of Clinical and Biological Sciences, San Luigi Gonzaga Hospital, University of Turin, Regione Gonzole 10, 10043, Orbassano, Turin, Italy.
Cilloni
Daniela
D
Division of Hematology and Internal Medicine, Department of Clinical and Biological Sciences, San Luigi Gonzaga Hospital, University of Turin, Regione Gonzole 10, 10043, Orbassano, Turin, Italy.
Saglio
Giuseppe
G
Division of Hematology and Internal Medicine, Department of Clinical and Biological Sciences, San Luigi Gonzaga Hospital, University of Turin, Regione Gonzole 10, 10043, Orbassano, Turin, Italy.
Guerrasio
Angelo
A
Division of Hematology and Internal Medicine, Department of Clinical and Biological Sciences, San Luigi Gonzaga Hospital, University of Turin, Regione Gonzole 10, 10043, Orbassano, Turin, Italy. angelo.guerrasio@unito.it.
D000368:Aged; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D005871:Castleman Disease / Q000188:drug therapy / Q000473:pathology / Q000821:virology; D018023:HIV Seronegativity; D019288:Herpesvirus 8, Human; D006801:Humans; D008297:Male; D012074:Remission Induction; D000069283:Rituximab; D012514:Sarcoma, Kaposi / Q000188:drug therapy / Q000473:pathology / Q000821:virology; D055502:Secondary Prevention
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D000069283:Rituximab