title: Clinical epidemiology and treatment patterns of patients with multicentric Castleman disease: results from two US treatment centres.
abstract: Multicentric Castleman disease (MCD) is a rare lymphoproliferative disease with little known about its epidemiology or treatment modalities. Clinical and demographic data of MCD patients identified between 2000 and 2009 were collected from medical records at two United States (US) MCD referral centres. ZIP codes identified patient residences; prevalence and incidence were estimated based on catchment areas. Patient clinical, demographic, and biochemical characteristics, drug therapies and medical utilization were descriptively reported. MCD patients (n = 59) were 61% male, mean age of 53 years (median = 55 years) and 68% Caucasian. Of those with known human immunodeficiency virus (HIV) status (n = 41), 85% (n = 35) were negative, 15% (n = 6) were positive. Most frequent physician-reported symptoms (n = 33) were fatigue (49%, n = 16), fever (39%, n = 13), and night sweats (30%, n = 10). The estimated US 10-year prevalence was 2·4 per million. During first year of follow-up after study entry, the top two systemic therapies (n = 27) were monotherapies: prednisone (33%, n = 9) and rituximab (19%, n = 5). After a follow-up of 2 years, 92% of patients were alive. This study provides new information on MCD population demographics, treatment patterns, and medical utilization; a minimal US period prevalence rate is proposed. Study replication is needed to improve external validity.
authors.affiliation: Janssen Global Services, Malvern, PA, USA.
authors.forename: Don
authors.initials: D
authors.lastname: Robinson
authors.forename: Matthew
authors.initials: M
authors.lastname: Reynolds
authors.forename: Corey
authors.initials: C
authors.lastname: Casper
authors.forename: Angela
authors.initials: A
authors.lastname: Dispenzieri
authors.forename: Jessica
authors.initials: J
authors.lastname: Vermeulen
authors.forename: Krista
authors.initials: K
authors.lastname: Payne
authors.forename: Judy
authors.initials: J
authors.lastname: Schramm
authors.forename: Kay
authors.initials: K
authors.lastname: Ristow
authors.forename: Marie-Pierre
authors.initials: MP
authors.lastname: Desrosiers
authors.forename: Karen
authors.initials: K
authors.lastname: Yeomans
authors.forename: Dana
authors.initials: D
authors.lastname: Teltsch
authors.forename: Richard
authors.initials: R
authors.lastname: Swain
authors.forename: Thomas M
authors.initials: TM
authors.lastname: Habermann
authors.forename: Philip
authors.initials: P
authors.lastname: Rotella
authors.forename: Helgi
authors.initials: H
authors.lastname: Van de Velde
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005871:Castleman Disease / Q000453:epidemiology* / Q000628:therapy; D005260:Female; D062306:Geography, Medical; D006295:Health Resources; D006801:Humans; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D014481:United States; D055815:Young Adult
keywords: Castleman disease; epidemiology; healthcare utilization; lymphoproliferative disorder; treatment