Archivos de Pediatría del Uruguay
Print version ISSN 0004-0584
GARCIA, CAROLINA et al. Enfermedad de Castleman en una adolescente. Arch. Pediatr. Urug. [online]. 2001, vol.72, n.4, pp. 288-292. ISSN 0004-0584.
Castleman`s disease (CD) is a B cell lymphoproliferative disorder, a rare disease of unknown cause. Although histologically is benign, a potential for malignancy has been described. There are two clinical types: localized (LCD) and multicentric (MCD); and three histologic variants: hyaline vascular, plasma-cell and mixed. The clinical presentation can vary from an asymptomatic localized mass to a systemic disorder with fever and constitutional symptoms.The optimal therapeutic aproach is still unknown. In the LCD complete surgical excision is usually curative, although the MCD is usually fatal with poor response to different treatment regimens.This report describes a case of CD in a fourteen-year- old girl who present a LCD in mediastinum with systemic symptoms and underwent a complete surgical excision. Laboratory values showed a remarkable plasmocytosis in the bone marrow aspirate. Both, laboratory data and clinical manifestation improve completely after surgery.
Keywords : GIANT LYMPH NODE HYPERPLASIA; MEDIASTINAL NEOPLASMS.