Nagata T, Kawamura N, Motoyama T, Miyake M, Yoden A, Yoshikawa K, Oguni T, Yamasiro K, Mino M
Department of Pediatrics, Hirakata City Hospital.
Jpn J Clin Oncol. 1992 Dec;22(6):421-7.
The case of a two-year-old girl with generalized histiocytosis, probably induced by phenobarbital, is reported. Symptoms, including intermittent fever, systemic lymphadenopathy, maculopapular skin eruption and hepatosplenomegaly, suggested Langerhans cell histiocytosis. Laboratory examinations revealed leukocytosis with lymphocytosis and eosinophilia and a high LDH serum level, while GOT and GPT were within normal ranges. Cytological studies of lymph node and pleural effusion specimens revealed proliferation and infiltration of Langerhans cell histiocytes with eosinophilia. No histiocyte proliferation was observed in the bone marrow or skin. The clinical manifestations shown by the patient were, however, transient, and improved spontaneously after the discontinuation of phenobarbital. The case was considered to be one of phenobarbital hypersensitivity syndrome based on clinical course and laboratory findings. The mechanism and differential diagnosis of the syndrome are discussed.
报告了一例可能由苯巴比妥诱发的两岁女童全身性组织细胞增多症病例。症状包括间歇性发热、全身淋巴结病、斑丘疹皮肤疹和肝脾肿大，提示朗格汉斯细胞组织细胞增多症。实验室检查显示白细胞增多伴淋巴细胞增多和嗜酸性粒细胞增多，血清乳酸脱氢酶水平升高，而谷草转氨酶和谷丙转氨酶在正常范围内。淋巴结和胸腔积液标本的细胞学研究显示朗格汉斯细胞组织细胞增生并伴有嗜酸性粒细胞浸润。骨髓或皮肤未观察到组织细胞增生。然而，患者表现出的临床表现是短暂的，停用苯巴比妥后自发改善。根据临床病程和实验室检查结果，该病例被认为是苯巴比妥过敏综合征之一。讨论了该综合征的机制和鉴别诊断。
