Clinical epidemiology and treatment patterns of patients with multicentric Castleman disease: Results from two US treatment centres

Don Robinson, Matthew Reynolds, Corey Casper, Angela Dispenzieri, Jessica Vermeulen, Krista Payne, Judy Schramm, Kay Ristow, Marie Pierre Desrosiers, Karen Yeomans, Dana Teltsch, Richard Swain, Thomas M. Habermann, Philip Rotella, Helgi Van de Velde

Research output: Contribution to journalArticle

35 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)39-48
Number of pages10
JournalBritish journal of haematology
Volume165
Issue number1
DOIs
StatePublished - Apr 2014

Keywords

  • Castleman disease
  • Epidemiology
  • Healthcare utilization
  • Lymphoproliferative disorder
  • Treatment

ASJC Scopus subject areas

  • Hematology

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    Robinson, D., Reynolds, M., Casper, C., Dispenzieri, A., Vermeulen, J., Payne, K., Schramm, J., Ristow, K., Desrosiers, M. P., Yeomans, K., Teltsch, D., Swain, R., Habermann, T. M., Rotella, P., & Van de Velde, H. (2014). Clinical epidemiology and treatment patterns of patients with multicentric Castleman disease: Results from two US treatment centres. British journal of haematology, 165(1), 39-48. https://doi.org/10.1111/bjh.12717