Predominant Stroma-Rich Feature In Hyaline Vascular Variant Of Castleman Disease Is Associated With Paraneoplastic Pemphigus

Objectives: We aimed to describe the clinical and histopathologic features of Castleman disease (CD), particularly emphasizing its associations with paraneoplastic pemphigus (PNP) and prognosis.Methods: We retrospectively enrolled 123 CD patients at our center. Clinical, pathologic and laboratory data were reviewed.Resuhs: Fifty percent of the patients had PNP. Compared with those without PNP, patients with PNP-associated CD had more hyaline vascular (HV) variants (83.9% vs 574%), fewer mixed cellular variants ( 16.1% vs 24.6%), and no plasmacytic variants (0% vs 18.0%). Thirtv-eight of 87 patients with the HV variant of CD ( HV-CD) had stroma-rich (SR) features and the incidence rate was higher in those with PNP-associated CD than in those without PNP (48.4% vs 13.1%, P < .001 ). The SR variant was associated with higher PNP-associated IgG titers than SR absence before surgery (median 1:160 vs 1:80, P = .019) or after surgery (median 1:160 vs 1:40, P = .013). The SR variant was also an unfavorable prognostic factor for CD survival in univariate analysis The 3-year survival rates were 47.5% among those with PNP and 87. 7% among those without PNP (P < .001).Conclusions: PNP is associated with specific subtypes of CD and affects survival. The SR variant of HV-CD positively correlates with the incidence of PNP. (C) American Society for Clinical Pathology, 2020. All rights reserved.