Kilpatrick S E, Wenger D E, Gilchrist G S, Shives T C, Wollan P C, Unni K K
Division of Anatomic Pathology, Mayo Clinic, Rochester, Minnesota, USA.
Cancer. 1995 Dec 15;76(12):2471-84. doi: 10.1002/1097-0142(19951215)76:12<2471::aid-cncr2820761211>3.0.co;2-z.
Langerhans' cell histiocytosis (LCH) of bone is a disorder of histiocytic proliferation with variable and often unpredictable behavior.
The authors evaluated the clinical and pathologic features of 263 patients (172 children, 91 adults) with biopsy-proven LCH examined during an 80-year period at the Mayo Clinic. Only patients with bone involvement pathologically and/or radiographically were included in the study. Clinical follow-up was available for 245 patients and ranged from 3 months to 50 years (mean, 12 years; median, 10 years). Chi-square tests were used to determine associations between age, gender, extent of osseous involvement, visceral disease, and pathologic features. Survival analyses were performed by univariate and multivariate Cox regression methods.
Age at presentation ranged from 2 months to 71 years with a clear predominance in children. The most common presenting complaint was pain, often worse at night. The skull was the most frequent osseous site in children and adults. Diabetes insipidus was documented in 40 patients. Forty-four children developed skeletal recurrence and/or new bone lesions, 19 of whom had diabetes insipidus. Fourteen children and 3 adults died either directly or indirectly from LCH. One adult patient developed systemic amyloidosis. All but two of these pediatric patients were 3 years of age or younger at presentation. All children with hepatosplenomegaly (7 patients) and/or thrombocytopenia (9 patients) died. Nine of the 14 children who died presented initially with three or more bone lesions.
The clinical behavior of LCH of bone is often unpredictable; however, young age at diagnosis, hepatosplenomegaly, thrombocytopenia, and polyostotic (> or = 3 bones involved) disease are associated with a poor prognosis (P < 0.005). Recrudescence in children, but not in adults, strongly correlates with the presence of diabetes insipidus (P < 0.0005).
骨朗格汉斯细胞组织细胞增多症（LCH）是一种组织细胞增殖性疾病，其行为多变且往往不可预测。
作者评估了梅奥诊所80年间经活检证实为LCH的263例患者（172例儿童，91例成人）的临床和病理特征。本研究仅纳入病理和/或影像学检查有骨受累的患者。245例患者有临床随访资料，随访时间为3个月至50年（平均12年；中位数10年）。采用卡方检验确定年龄、性别、骨受累程度、内脏疾病和病理特征之间的关联。通过单因素和多因素Cox回归方法进行生存分析。
发病年龄为2个月至71岁，儿童明显居多。最常见的主诉是疼痛，通常夜间加重。颅骨是儿童和成人最常受累的骨部位。40例患者有尿崩症记录。44例儿童出现骨骼复发和/或新的骨病变，其中19例有尿崩症。14例儿童和3例成人直接或间接死于LCH。1例成年患者发生系统性淀粉样变性。这些儿科患者中除2例外，其余均在发病时年龄为3岁或更小。所有有肝脾肿大（7例）和/或血小板减少症（9例）的儿童均死亡。14例死亡儿童中有9例最初表现为三个或更多骨病变。
骨LCH的临床行为往往不可预测；然而，诊断时年龄小、肝脾肿大、血小板减少症和多骨受累（累及≥3块骨）疾病与预后不良相关（P<0.005）。儿童复发，但成人不复发，与尿崩症的存在密切相关（P<0.0005）。
