Fever and Skin Involvement at Diagnosis Predicting the Intractable Langerhans Cell Histiocytosis: 40 Case-Series in a Single Center.
Fever and Skin Involvement at Diagnosis Predicting the Intractable Langerhans Cell Histiocytosis: 40 Case-Series in a Single Center.
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诊断时的发热和皮肤受累可预测顽固性朗格汉斯细胞组织细胞增多症：单个中心的 40 个病例系列。
DOI：
10.1097/mph.0000000000001080
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发表时间：
2018
期刊：
影响因子：
--
通讯作者：
Ohga S
中科院分区：
文献类型：
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作者：
Kobayashi T;Koga Y;Ishimura M;Nakashima K;Kato W;Ono H;Sonoda M;Eguchi K;Fukano R;Honjo S;Oda Y;Ohga S
朗格汉斯细胞组织细胞增多症（LCH）是一种免疫反应不明的克隆性疾病。LCH患者偶尔会出现发热，但其意义尚不明确。我们研究了难治性和/或再激活性LCH发生难治性疾病的预测因素。共纳入1998 - 2014年在九州大学管理的40例小儿LCH患者。对病历进行回顾性分析。16例患者存在累及危险器官（ROs）的多系统（MS） LCH （n= 4）或未累及危险器官（n= 12）。共有24例患者发生单系统LCH，累及骨骼（多系统8例，单系统13例）、皮肤（2例）或淋巴结病变（1例）。8例难治性疾病7例多发性硬化或1例多骨LCH。2例患者死于伴或不伴RO累及的MS LCH。10例患者发病时出现持续发热（bb0 - 38℃）。难治性病例有发热、RO及皮肤受累、白细胞增多、凝血功能障碍、小细胞性贫血、可溶性白细胞介素-2受体和c反应蛋白水平增高，诊断时较为常见。多因素分析显示，诊断时发热和皮损与顽固性独立相关（优势比：发热35.5；95%可信区间3.0-1229.1；皮损24.6；95%可信区间1.9-868.7）。即使没有RO累及，最初的发热和皮肤累及也可能预示难治性和致命危险LCH的发展。
Langerhans cell histiocytosis (LCH) occurs as a clonal disease with enigmatic immune responses. LCH patients occasionally present with fever, although the significance remains elusive. We investigated the predicting factors for developing intractable disease of refractory and/or reactivated LCH. In total, 40 pediatric LCH patients managed in Kyushu University from 1998 to 2014 were enrolled. The medical records were analyzed retrospectively. Sixteen patients suffered from multisystem (MS) LCH involving risk organs (ROs)(n= 4) or not (n= 12). In total, 24 patients had single-system LCH affecting bone (multi n= 8, single n= 13), skin (n= 2), or lymph node lesions (n= 1). Eight patients had the intractable disease of 7 MS or 1 multibone LCH. Two patients died from MS LCH with or without RO involvement. Ten patients showed persistent fever (> 38 C) at onset. Intractable cases had fever, RO and skin involvement, leukocytosis, coagulopathy, microcytic anemia, higher levels of soluble interleukin-2 receptor and C-reactive protein, more frequently at diagnosis. Multivariate analysis indicated that fever and skin lesions at diagnosis were independently associated with the intractability (odds ratio: fever, 35.5; 95% confidence interval, 3.0-1229.1; skin lesions, 24.6; 95% confidence interval, 1.9-868.7). Initial fever and skin involvement might predict the development of intractable and fatal-risk LCH even without the RO involvement.