The clinical and pathological subtypes of Castleman's disease and their relationship with complications: a large series analysis from a single center / 中华血液学杂志

ABSTRACT

<p><b>OBJECTIVES</b>To investigate the clinical and pathological subtypes of Castleman's disease (CD) and their relationship with complications.</p><p><b>METHODS</b>The clinical complications of 53 patients with CD and the relationship of these complications with clinical and pathological subtypes were analyzed retrospectively.</p><p><b>RESULTS</b>Among 53 CD patients, 32 (60.4%) were classified as uni-centric type and 21 (39.6%) multicentric type. Histopathological examination showed that 37 cases (69.8%) were hyaline vascular variants (HV), 9 (17.0%) plasmacytic variants (PC), and 7 (13.2%) mixed cellular variants (Mix). Complications were identified in 32 (60.4%) patients, including the involvements of skin, internal organs and hematopoietic system. Some complications were closely associated with the clinical subtype of CD the majority of complications in the 32 uni-centric CDs were paraneoplastic pemphigus (PNP) and bronchiolitis obliterans (BO), and those in 21 multi-centric CDs were the involvements of kidney and hematopoietic system. The complications were different among the three kinds of histopathological subtypes PNP and BO were the predominant complications of HV variants, while the internal organ and hematopoietic system involvements were those of PC and Mix variants. The clinical and histopathological classification of CD patients with PNP were different obviously from other subtypes of CDs. In Kaplan-Meier survival analysis, the survival rate of those with complications was significantly lower than those without complication (P = 0.028).</p><p><b>CONCLUSION</b>The clinical complications of CDs are related to their clinical and histopathological subtypes. CD patients with PNP should be considered as a unique entity to tailor the therapy. The presence of clinical complications is an independent prognostic factor in CD patients.</p>