Clinical characteristics of 5 children with Castleman's disease and review of literature / 中华儿科杂志

ABSTRACT

<p><b>OBJECTIVE</b>To investigate the clinical characteristics of children with Castleman's disease and to improve doctors' awareness of this disease.</p><p><b>METHODS</b>Clinical characteristics of 5 cases with Castleman's disease were observed and analyzed and relevant reports in literature were reviewed.</p><p><b>RESULTS</b>(1) All the five patients' histories were long, and the first symptoms of them were painless lymphnode enlargement, and all of them were at school age; 3 patients' abdominal lymphnodes were enlarged, mediastinum lymphnodes enlarged in 3 cases, cervical lymphnodes were involved in 3 cases; (2) The clinical subtypes the disease in 3 cases was localized Castleman's disease (LCD), all of their pathological subtype was hyaline vascular variant (HV). The rest of them were multicentric Castleman's disease (MCD), whose pathology was plasma cell variant (PC), and both of them had a febrile symptoms; (3) The white blood cells, C-reactive protein and ferritin levels were all elevated to different extents. Four of them had viral infections, and their cellular immune function was abnormal; (4) The LCD patients' prognosis was good after the complete resection. There is no standard therapy for MCD, the available therapies include antiviral, immune modulatory regimens, CD20 B cell monoclonal antibody and chemotherapy, but the prognosis was worse than that of LCD.</p><p><b>CONCLUSIONS</b>Castleman's disease is rare in children, which can be misdiagnosed because it has no specific manifestations. The prognosis depends on the subtype.</p>