title: Clinical Features and Outcome in HIV-Associated Multicentric Castleman's Disease.
abstract: OBJECTIVE
  To describe clinical features, treatment outcomes and relapse rates in HIV-associated multicentric Castleman's disease (MCD) in a sizeable mature cohort.
  
  
  METHODS
  From a prospective database, we identified 61 HIV-seropositive patients with histologically confirmed MCD (median follow-up, 4.2 years). Since 2003, 49 patients with newly diagnosed MCD have been treated with rituximab with (n = 14) or without (n = 35) etoposide.
  
  
  RESULTS
  At MCD diagnosis, 55 (90%) of 61 patients met proposed clinical criteria defining an attack. Four patients (7%) had histologic evidence of coexisting lymphoma, and one developed lymphoma 2 years after treatment. The incidence of lymphoma is 28 per 1,000 patient years. With rituximab-based treatment, the overall survival was 94% (95% CI, 87% to 100%) at 2 years and was 90% (95% CI, 81% to 100%) at 5 years compared with 42% (95% CI, 14% to 70%) and 33% (95% CI, 6% to 60%) in 12 patients treated before introduction of rituximab (log-rank P < .001). Four of 49 rituximab-treated patients have died; three died as a result of MCD within 10 days of diagnosis, and one died as a result of lymphoma in remission of MCD. Eight of 46 patients who achieved clinical remission suffered symptomatic, histologically confirmed MCD relapse. The median time to relapse was 2 years, and all have been successfully re-treated and are alive in remission. The 2- and 5-year progression-free survival rates for all 49 patients treated with rituximab-based therapy were 85% (95% CI, 74% to 95%) and 61% (95% CI, 40% to 82%), respectively.
  
  
  CONCLUSIONS
  HIV-associated MCD is a remitting-relapsing disease. The outlook has improved dramatically in recent years with the introduction of rituximab-based therapy and yields high overall survival rates.
authors.affiliation: Department of Oncology, Imperial College School of Medicine, The Chelsea and Westminster Hospital, 369 Fulham Rd, London SW10 9NH, UK. m.bower@imperial.ac.uk
authors.forename: Mark
authors.initials: M
authors.lastname: Bower
authors.forename: Tom
authors.initials: T
authors.lastname: Newsom-Davis
authors.forename: Kikkeri
authors.initials: K
authors.lastname: Naresh
authors.forename: Shairoz
authors.initials: S
authors.lastname: Merchant
authors.forename: Belinda
authors.initials: B
authors.lastname: Lee
authors.forename: Brian
authors.initials: B
authors.lastname: Gazzard
authors.forename: Justin
authors.initials: J
authors.lastname: Stebbing
authors.forename: Mark
authors.initials: M
authors.lastname: Nelson
mesh_terms: D000328:Adult; D000368:Aged; D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage / Q000627:therapeutic use; D005871:Castleman Disease / Q000188:drug therapy / Q000453:epidemiology / Q000821:virology; D015897:Comorbidity; D016208:Databases, Factual; D018450:Disease Progression; D018572:Disease-Free Survival; D004359:Drug Therapy, Combination; D005047:Etoposide / Q000008:administration & dosage / Q000627:therapeutic use; D005260:Female; D015658:HIV Infections / Q000453:epidemiology*; D006566:Herpesviridae Infections / Q000453:epidemiology; D019288:Herpesvirus 8, Human / Q000302:isolation & purification; D006801:Humans; D015994:Incidence; D053208:Kaplan-Meier Estimate; D016483:Lymphoma, AIDS-Related / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D016016:Proportional Hazards Models; D000069283:Rituximab; D016019:Survival Analysis; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab; D005047:Etoposide