Pediatric idiopathic multicentric Castleman disease presenting with massive ascites and pleural effusions

Abstract

Castleman disease is a rare lymphoproliferative disorder with varying clinical presentations. In pediatric populations, the incidence of idiopathic multicentric Castleman disease (iMCD) is exceedingly rare, with literature limited to isolated case reports and case series. Herein, we describe a 14-year-old female who presented with massive ascites, pleural effusions, and diffuse lymphadenopathy found to have iMCD. This unusual presentation of pediatric iMCD is similar to adult TAFRO subtype, but without severe thrombocytopenia. After a limited treatment course of rituximab and prednisone, the patient achieved complete remission.