Nationwide Survey of Single-System Single Site Langerhans Cell Histiocytosis in Japan
Nationwide Survey of Single-System Single Site Langerhans Cell Histiocytosis in Japan
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DOI：
10.1002/pbc.22224
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发表时间：
2010-01-01
影响因子：
3.2
通讯作者：
Ishii, Eiichi
中科院分区：
文献类型：
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作者：
Morimoto, Akira;Ishida, Yasushi;Ishii, Eiichi
背景。由于目前没有针对单系统单部位（SS-s）型朗格汉斯细胞组织细胞增多症（LCH）的标准治疗和方案研究，我们在日本进行了一项全国性调查，以阐明该亚型的流行病学和临床结果。过程。关于1995年至2006年间诊断为ss -s型LCH的儿童的临床病程的问卷发送给日本儿科血液学学会的所有成员。结果。146例经组织学证实的SS-s型LCH患儿可评价。最常受影响的器官是骨骼（82%），其次是皮肤（12%）。少数患者（14%）有组织细胞学会定义的中枢神经系统风险病变。皮肤病变患者的诊断年龄明显小于骨病变患者(中位：6个月vs. 5年11个月，P
Background. Since neither a standard treatment nor a protocol study for single-system single site (SS-s)-type Langerhans cell histiocytosis (LCH) exists, we conducted a nationwide survey in Japan to clarify the epidemiology and clinical outcome of this subtype. Procedure. Questionnaires regarding the clinical course of children with SS-s-type LCH diagnosed between 1995 and 2006 were sent to all members of the Japanese Society of Pediatric Hematology. Results. One hundred forty-six children with histologically proven SS-s LCH were evaluable. The most frequently affected organ was bone (82%), followed by skin (12%). Few patients (14%) had a CNS-RISK lesion defined by the Histiocyte Society. Patients with a skin lesion were diagnosed at a significantly younger age than patients with a bone lesion (median: 6 months vs. 5 years 11 months, P