Parez N, Bader-Meunier B, Roy C C, Dommergues J P
Service de Pédiatrie, CHU Bicêtre, Le Kremlin Bicêtre, France.
Eur J Pediatr. 1999 Aug;158(8):631-7. doi: 10.1007/s004310051166.
Castleman disease is a distinct lymphoproliferative disorder of unknown origin. Seven new cases in children are reported here and 76 cases from the paediatric literature are reviewed. The disease has been reported in 46 females and 37 males, their age ranging from 2 months to 17 years. The disease was localized in 72 cases and multicentric in 11 cases. The hyalinovascular type was more frequently encountered (54%) than the plasma cell type (24%). Laboratory abnormalities were more often associated with the plasma cell type and were mainly represented by anaemia and hypergammaglobulinaemia. Treatment of the localized tumour consisted of surgical excision, whereas treatment of the multicentric form was medical, comprising prednisone and other immunosuppressor drugs. The disease in the paediatric population seems to have a more favourable course than in adults.
The paediatric features of the disease suggest that Castleman disease in this population could represent an earlier form of the pathology or even suggest a benign lymphoproliferative disorder.
卡斯特尔曼病是一种病因不明的独特的淋巴增生性疾病。本文报告了7例儿童新病例，并复习了儿科文献中的76例病例。该病已报告46例女性和37例男性，年龄范围为2个月至17岁。72例为局限性，11例为多中心性。透明血管型比浆细胞型更常见（54%），浆细胞型占24%。实验室异常更多与浆细胞型相关，主要表现为贫血和高球蛋白血症。局限性肿瘤的治疗包括手术切除，而多中心型的治疗为药物治疗，包括泼尼松和其他免疫抑制药物。儿科人群中的该病似乎比成人有更有利的病程。
该病的儿科特征表明，该人群中的卡斯特尔曼病可能代表了该病理的早期形式，甚至提示为良性淋巴增生性疾病。
