Clinical and pathological characteristics of Castleman disease: an observational study in a Spanish tertiary hospital. Issue 14 (6th December 2019)

• Record Type: Journal Article
• Authors: González-García, Andrés; Patier de la Peña, José Luis; García-Cosio, Mónica; Sarhane, Yasmina; Sánchez Díaz, Cristina; Barbolla Díaz, Ignacio; López Rodríguez, Mónica; Moreno, María Ángeles; Villarubia, Jesús; Manzano, Luis
• Is Part Of: Leukemia & lymphoma. Volume 60:Issue 14(2019)
• Page Start: 3442
• Page End: 3448
• Publication Date: 2019-12-06
• Subjects: Unicentric Castleman disease -- multicentric Castleman disease -- human herpesvirus-8 -- Kaposi sarcoma -- interleukin-6 -- human immunodeficiency virus; Leukemia -- Periodicals; Lymphomas -- Periodicals; 616.99419
• DOI: 10.1080/10428194.2019.1639168
• Languages: English
• ISSNs: 1042-8194

Abstract

Abstract: Castleman disease (CD) represents a heterogeneous group of lymphoproliferative disorders that share well-defined histopathological features. An observational study of patients with CD was conducted. A total of 53 patients had CD: 20 had the unicentric form (UCD) and 33 the multicentric (MCD) variant; 10 of the latter cases were infected with human herpesvirus-8 (HHV-8) and 23 were idiopathic (iMCD). Median age differed between UCD and iMCD (30 vs. 49 years, p = .004). Males were completely predominant in HHV-8-associated MCD (100%), and females were more frequent in UCD (75 vs. 48%, p = .06). Relapses were more frequent in iMCD (57 vs. 10% UCD, p = .002), and mortality was significantly higher in iMCD and the HHV-8-associated form with respect to UCD. We conclude that UCD is a benign disorder of younger ages and female predominance, while iMCD represents a different entity with more disease relapses and higher mortality.