Martinez-Perez D, Mulliken J B, Arceci R J
Division of Plastic Surgery, Children's Hospital, Boston, Massachusetts, USA.
Plast Reconstr Surg. 1996 Aug;98(2):211-6. doi: 10.1097/00006534-199608000-00002.
Langerhans cell histiocytosis is an infrequent and enigmatic proliferative disorder that commonly presents in the head and neck region. This is an analysis of 77 patients with Langerhans cell histiocytosis treated at Children's Hospital and Dana Farber Cancer Institute from 1974 through 1993. The study focused on clinical findings, anatomic location and extent of disease, therapy, and outcome. The patients were, on average, under 5 years of age at initial presentation. Over 62 percent of the patients had signs and symptoms referred to the craniofacial skeleton. Osteolytic lesions of the cranium were the most common, followed, in frequency, by scalp rash, osteolytic mandibular tumor(s), enlarged nodes, and gingival swelling or ulceration. Single bony lesions usually were treated with curettage or radiotherapy. Chemotherapy was used commonly for advanced disease with multifocal or disseminated presentation. Initial therapy included moderate doses of single agents; other agents were added if no response was achieved. The natural history of Langerhans cell histiocytosis varied from an acute fulminant course, a waxing and waning chronic disease, to spontaneous regression. Young age at presentation and organ dysfunction predicted a poor prognosis. Statistical analysis showed that there was no significant relationship between outcome and extent of skeletal involvement when controlling for age or organ dysfunction.
朗格汉斯细胞组织细胞增多症是一种罕见且神秘的增殖性疾病，常见于头颈部区域。本文分析了1974年至1993年在儿童医院和丹娜法伯癌症研究所接受治疗的77例朗格汉斯细胞组织细胞增多症患者。该研究聚焦于临床发现、疾病的解剖位置和范围、治疗方法及治疗结果。患者初诊时平均年龄不到5岁。超过62%的患者有涉及颅面骨骼的体征和症状。颅骨的溶骨性病变最为常见，其次依次为头皮皮疹、下颌骨溶骨性肿瘤、淋巴结肿大以及牙龈肿胀或溃疡。单个骨病变通常采用刮除术或放射治疗。对于多灶性或播散性表现的晚期疾病，通常采用化疗。初始治疗包括中等剂量的单一药物；若未取得疗效，则加用其他药物。朗格汉斯细胞组织细胞增多症的自然病程各异，从急性暴发性病程、病情波动的慢性疾病到自发缓解。初诊时年龄小和器官功能障碍预示预后不良。统计分析表明，在控制年龄或器官功能障碍的情况下，治疗结果与骨骼受累程度之间无显著关联。
