Published online Feb 6, 2019. doi: 10.12998/wjcc.v7.i3.373
Peer-review started: October 30, 2018
First decision: November 14, 2018
Revised: November 26, 2018
Accepted: December 12, 2018
Article in press: December 12, 2018
Published online: February 6, 2019
Castleman disease (CD) is a rare lymphoproliferative disorder that presents with various symptoms. CD accompanied with jaundice is uncommon since there are only 11 cases reported in the literature.
Here we report a 62-year-old woman who was admitted to the hospital with signs and symptoms of intermittent jaundice. Biochemical tests showed higher serum levels of total and direct bilirubin, and normal serum levels of tumor markers and interleukin-6. Contrast-enhanced computed tomography detected a 6 cm × 4 cm × 2.5 cm mass between the hepatoduodenal ligament and the inferior vena cava. The mass was successfully excised and the patient had a complete resolution of symptoms. A diagnosis of idiopathic unicentric CD was made based upon histological examination, which demonstrated the pathological features of CD of mixed type, including hyperplasia of follicular lymphoids with abundant plasma cells, degenerative germinal centers, interfollicular vascularity and hyaline degeneration. The diagnosis was corroborated by immunohistochemical analysis which detected multiple biomarkers.
This is the first study that describes the clinicopathological features of CD presenting with jaundice, which may deepen and extend our understanding of this disease.
Core tip: Castleman disease (CD) is a rare lymphoproliferative disorder that presents with various symptoms. Here we report an uncommon case of CD accompanied with intermittent jaundice caused by extrinsic compression of a mass located between the hepatoduodenal ligament and the inferior vena cava. Histological examination demonstrated the pathological features of idiopathic unicentric CD of mixed type, supported by the immunohistochemical detection of multiple markers. The clinicopathological and immunohistochemical study on the case of CD provides new insights and diagnostic criteria of this orphan disease.