Clinical, laboratory and imaging findings in Castleman's disease - The subtype decides.

Castleman's disease (CD) is a rare lymphoproliferative disorder with its distinct unicentric (uCD) and multicentric (mCD) entities. The present work aimed at characterizing CD in more detail. From the 775 articles found by a PubMed search, 1133 cases were extracted. Two own cases were included. UCD was identified in 719 (42% males) and mCD in 416 (63% males) cases. Age in uCD was 34±17 and in mCD 48±18years. The hyaline-vascular type predominated in uCD and the plasma cell type in mCD. Clinical symptoms were more common in mCD. The head and neck region was most frequently affected in uCD and the axillary region in mCD. Prevalence of human immunodeficiency virus (HIV) and human herpesvirus-8 (HHV-8) positivity was higher in mCD. In CT scans, high contrast enhancement and calcifications were more frequent in uCD (all p<0.0001). The two forms of CD not only differ markedly in their clinical, laboratory and imaging findings, but also in treatment response and prognosis.