骨朗格汉斯细胞组织细胞增生症25例临床分析

Clinical analysis of 25 cases with Langerhans-cell histiocytosis in bone

目的分析骨朗格汉斯细胞组织细胞增生症（LCH）的临床特点，总结LCH诊断和治疗的方法。方法回顾性分析2004年2月至2012年2月收治的25例经病理证实的LCH患者的临床资料及随访结果。结果25例患者中男18例，女7例；中位年龄17岁。单发病灶17例，其中颅骨11例，多发病灶6例。首发症状多为疼痛和局部肿块，全身症状少见。主要症状多为局部疼痛，影像学表现为溶骨性改变，12例伴周围软组织肿胀。病理表现为分化好的组织细胞增生及大量嗜酸性粒细胞浸润，CD1a、S100、Vimentin、CD68免疫组织化学阳性率高。单发病例采用手术治疗为主，辅以放疗或化疗。多发病例以化疗为主，辅以放疗。仅累及骨的患者疗效满意，累及其他脏器的2例患者死亡。结论骨LCH男性发病明显高于女性，好发于儿童和青少年，以单发病灶为多，颅骨侵犯多见。临床表现主要为局部疼痛和肿块，病理活组织检查是确诊的首选方法，治疗宜采用综合疗法。预后与骨病损范围和病理类型以及其他脏器受累情况，大部分病例预后好。

Objective To investigate the clinical characteristics of bone Langerhans-cell histiocytosis （LCH） and evaluate its diagnosis, therapy and prognosis. Methods 25 cases with biopsy confirmed bone LCH during the last 8 years were retrospectively analyzed. Results The patients included 18 males and 7 females, 13 children and 12 adults, ranging from 1.5 to 55 years old with a median age of 17. Cases with unifocal lesions were 17, including 11 cases of skull LCH, and the remaining 8 were with multifocal lesions. First symptoms were predominantly pain and local masses, and rarely constitutional symptoms. The manifestation of radiography was osteolytic bony lesions. 12 cases had masses in soft tissues. Patients with solitary lesions underwent surgical operation, followed by radiotherapy or chemotherapy. Cases with muhifocal lesions received chemotherapy and radiotherapy. Pathological examination showed proliferation of well differentiated histiocytes, and large numbers of infiltrating eosinophils. Positive rates of CDla, S100, Vimentin and CD68 were higher in immunohistochemistry. Patients with restricted involvement in bones can achieve a satisfactory therapeutic effect. 2 cases died when multiple systems were compromised. Conclusion Bone LCH occurs predominantly in children and teenagers, involves solitary bones,and morbidities in males are much higher than females. Skull is most commonly affected. Principal clinical manifestations are pain and local masses. Diagnosis of bone LCH depends on microscopic examination. Combination therapy appears to be an effective method of treatment. Prognosis of disease is related to the degree of bone involvement, histological classification and simultaneously encroachment of other organs. Most patients have good prognosis.