Resumo

CASTILLETTI H, Jorge M; VARGAS S, Denny M; MARTINEZ, Marta  e  ROJAS, Nilda. Enfermedad de castleman: A propósito de dos casos. Arch Venez Puer Ped [online]. 2008, vol.71, n.2, pp.54-58. ISSN 0004-0649.

Castleman's disease or angiofolicular hiperplasy is characterized by reactive lymphoid hyperplasia, benign tumors of lymphoid tissue and predisposition to develop lymphomas. Described by Benjamin Castleman in 1956, it is of unknown etiology, probably related to herpes virus type 8, immunoregulation failure, increased expression of 6-interleukin gene. Very rare in childhood, the disease has two different clinical types: a multicentric type, and a localized type (70% of the cases). The latter with good prognosis, located in mediastinum, neck, abdomen, and less frequently in axila, pelvis and pancreas. Treatment consists in the resection of the lesion. The histological types are the hyaline-vascular type (most frequent) and the plasma cells type. Literature was reviewed and two clinical cases are reported: Case nº 1: 6 year old child, who presents at 18 months of age with a 0.5 cm bulk in his left axila. The lesion was removed surgically at 4 years of age, with reappearance of a 7 x 4 lesion which was removed at 6 years of age. This child had also hipergammaglobulinemia, low levels of NK cells and of the CD4/CD8 index. Case nº 2: 4 year old child, who presented with a 1 cm mass in his right axila of 6 months of evolution. An excisional biopsy was performed. In both cases the the histological study reported Castleman’s disease of hialinovascular variety. Pediatricians and pediatric surgeons must follow very closely the growth of lymphoid tissue that may represent immunological. or neoplastic lesions, potentially curable if diagnosed and treated early.

Palavras-chave : Castleman's disease; lymphoid hyperplasia; hyaline-vascular type; benign tumor.