Unicentric castleman disease is not clearly distinguished from multicentric type: a case report

Castleman disease (CD) was recently classified as a unicentric hyaline vascular variant, unicentric plasma cell variant, and multicentric plasma cell variant. It is rare that unicentric CD is presented as multiple retroperitoneal lymphadenopathy. The clinical manifestations and prognosis depends on histologic type. We report an unusual case of CD with multiple retroperitoneal lymphadenopathy, which had unicentric hyaline vascular variant histologically but was clinically multicentric. The patient experienced anemia, weight loss, elevated C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR), POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome), and plasmacytosis in bone marrow without human herpesvirus-8 or HIV. After exploratory laparotomy and lymphadenectomy under presumptive diagnosis of CD, the patient's symptoms recovered, and CRP and ESR decreased. Therefore, we suggest that unicentric CD is not clearly distinguished from multicentric, the type in this report, focusing on the useful role of CRP, ESR, and positron emission tomography/computed tomography in the disease activity of CD.