颞骨朗格汉斯组织细胞增生症22例临床分析

Langerhans cell histiocytosis of the temporal bone： 22 cases analysis

目的分析颞骨朗格汉斯组织细胞增生症（Langerhans cell histioeytosis，LCH）的临床表现、诊断、治疗及预后。方法回顾性分析我院15年来收治的22例颞骨LCH，研究其临床表现、诊断、治疗及预后，并随访1～15年，进行生存分析和无病生存率的Log—rank检验。结果22例中1例为多系统高危组，3例为多系统低危组，18例为单系统组。临床表现以耳部为主，颞骨CT见大范围的溶骨性骨质破坏。22例患者的误诊率为72．7％（15／22）。所有患者采用不同组合的综合治疗，随访1～15年，存活21例（95．5％），死亡1例（4．5％），后遗尿崩症、侏儒症、癫疴各1例，单耳听力严重下降2例，后遗症发生率22．7％（5／22），多系统组与单系统组无病生存曲线差异有统计学意义（Х^2=5．87，P〈0．05）。结论颞骨LCH多数累及单系统，以耳部临床表现为主，易误诊，治疗方案宜根据受累系统来选择，单系统组预后明显好于多系统组。

Objective To analyze the clinical manifestations, diagnosis, therapy and prognosis of Langerhans cell histiocytosis（LCH） of the temporal bone. Methods Twenty-two patients with LCH of the temporal bone in our hospital were retrospectively summed up from January 1994 to January 2008. Following up 1- 15 years, their clinical manifestations, diagnosis, therapy and prognosis were studied. Survival analysis and disease free survival Log-rank test were used. Results Among 22 patients, 1 case belonged to the multisystem high risk group, 3 cases to the multisystem low risk group, and 18 cases to the single system group. The clinical characters were primarily the ear presentations, CT of the temporal bones showed extensive osteolytic destructions. The misdiagnosis rate was 72.7%. Twenty-two cases were received different combined modality therapies. Following up 1 - 15 years, 21 cases were survival and 1 case dead （4. 5% ）, while 5 cases showed residual of insipidus, dwarfism, epilepsy or unilateral serious hearing loss （22. 7% ）. For disease free survival curve, there was a significant difference between the multisystem group and the single system group （Х^2 = 5.87, P 〈 0. 05 ）. Conclusions As LCH of the temporal bone, the single system cases are predominant. The ear area clinical manifestations arc primary. This disease is easily misdiagnosed. The therapy selection should rely on the involved systems of the disease. The prognosis of the single system group is much better than the muhisystem group.