| Literature DB >> 34137908 |
Sevtap Tugce Ulas1, Süha Dasdelen2,3.
Abstract
Castleman disease (CD) is a very rare disorder characterised by hyperplasia of the lymphoid tissue. The aetiology varies considerably and includes autoimmunological, infectious, autoinflammatory and paraneoplastic diseases (e.g. MGUS with POEMS syndrome). What they all have in common is usually a dysregulation/overproduction of certain cytokines and growth factors (including interleukin 6 and VEGF). The sum of these changes sometimes causes very heterogeneous symptoms and thus often makes early diagnosis difficult. The prognosis of unrecognised and untreated disease is very serious and has an average 5‑year survival rate of 55-77%. The present paper describes the case of a 79-year-old patient with refractory polyserositis who was correctly diagnosed after > 8 years.Entities:
Keywords: Antibodies, monoclonal; Interleukin‑6; Monoclonal gammopathy of undetermined significance; Multicentric Castleman’s disease; Vascular endothelial growth factor (VEGF)
Year: 2021 PMID: 34137908 DOI: 10.1007/s00108-021-01063-4
Source DB: PubMed Journal: Internist (Berl) ISSN: 0020-9554 Impact factor: 0.743