摘要
目的 探讨朗格汉斯细胞组织细胞增生症（LCH）的临床特征.方法 回顾分析2例LCH患者的临床资料并复习相关文献.结果 2例均为未成年男性患儿颅骨受累伴骨质破坏,1例为单发灶累及眶后壁导致突眼,另1例为多发灶导致额部及枕部多发包块,2例均经病理学证实为LCH（其中1例为嗜酸性肉芽肿）.第1例给予病灶切除术加放疗,第2例未行放化疗.结论 LCH是一种综合征,头颈部受累很常见,病灶定位于颅骨时可表现为头部包块、眼球突出等,临床上出现上述症状时应考虑LCH可能.确诊需要病理学及免疫组织化学证据.治疗根据临床表现及受累器官选择治疗方案.预后与受累器官数目和功能受损情况密切相关.
Objective To explore the clinical features of Langerhans＇cell histiocytosis （LCH）.Methods The clinical data of 2 cases of LCH were retrospectively analyzed in addition with review of literatures.Results 2 cases of LCH were both children and males with skull involvement.One with a single lesion that involved the posterior orbit presented as proptosis,another with multiple lesions presented as several skull masses.Both were confirmed as LCH by pathology （one was Eosinophilic granuloma）.Case 1 was given total excision of the lesion and radiotherapy,case 2 had no treatment.Conclusion LCH is a kind of clinical syndrome and commonly affects the head and neck.Patients with cranial bones involvement usually present as skull masses,proptosis,etc.Clinically when these symptoms appears,the diagnosis of LCH should be under consideration.Definitive diagnosis of LCH requires pathological and immunohistochemical confirmation.The choice for its treatment should be based on clinical manifestations and the involved organs.Its prognosis may be associated with the number of involvement of organs and impairment of function.
出处
《临床内科杂志》
CAS
2013年第10期694-696,共3页
Journal of Clinical Internal Medicine
