伴发中枢性尿崩症的郎格罕细胞组织细胞增生症3例

郎格罕细胞组织细胞增生症(Langerhans cell histiocytosis，LCH)，曾被称为组织细胞增生症X，是一种主要发生在儿童和青少年的系统性疾病。LCH最常见的受累器官是骨骼和皮肤，少数也可累及中枢神经系统，特别是下丘脑-垂体区(hypothalamus-pituitary region, HPR)，引起中枢性尿崩症(central diabetes insipidus, CDI)。我院血液肿瘤科近年来收治了3例伴发CDI的LCH 患者，其中2例以CDI为首发症状，现予报道并讨论此类疾病诊治情况。     

郎格罕细胞组织细胞增生症并发脏器功能衰竭的特点和危险因素

分析34例郎格罕细胞组织细胞增生症并发脏器功能衰竭13例，其特点为：≤2岁年龄组多发，衰竭器官以肺脏、血液、肝脏为主，多器官衰竭者均死亡。危险因素有：①呼吸衰竭；②感染；③并存疾病存在，特别是肺炎，营养不良者；④酸中毒；⑤年龄≤2岁。认为≤2岁的勒雪氏病是防治的重点人群，积极抗炎，防止呼衰，监测器官功能，营养支持是防止器官功能衰竭的中心环节。     

郎格罕细胞组织细胞增生症临床诊治的新进展

郎格罕细胞组织细胞增生症临床诊治的新进展周伟孙怀珍郎格罕细胞组织细胞增生症（ｌａｎｇｅｒ－ｈａｎｓｃｅｌｈｉｓｔｉｏｃｙｔｏｓｉｓ，ＬＣＨ）可表现为能自行缓解的单一骨损害到威胁生命的多系统受累等多种形式。过去１０年病理和医学影像学的进展，使临床和病理...     

郎格罕细胞组织细胞增生症肺部病变临床特点分析

为探讨郎格罕细胞组织细胞增生症(LCH)患儿肺部病变临床特点，对20例郎格罕细胞组织细胞增生症患儿进行分析，其中男12例，女8例；就诊年龄4个月～6岁6个月。结果显示20例患儿均具有间质性肺损害，但出现呼吸道症状者仅占57％。有阵发性咳嗽ll例，气促、呼吸衰竭1例，反复呼吸道感染1例，无咯血、气胸、紫绀者；肺部体征有呼吸音粗糙6例，肺部罗音1例；活动期患儿可有血气和肺功能异常；X线异常多出现在呼吸道症状之前，其中双肺纹理粗多ll例，肺门影增粗模糊2例，网点状阴影10例，片状影10例，肺囊泡5例，毛玻璃样1例，肺气肿1例，胸膜增厚1例，胸腺肿大2例。提示胸部高分辨率CT扫描有利于早期发现LCH的肺部改变；早期、全身、强化疗有利于肺部病变的恢复。     

郎格罕细胞组织细胞增生症的病理诊断

探讨病理检查在郎格罕细胞组织细胞增生症的诊断中的作用。收集我院1991-2001年间收治的诊断为小儿郎格罕细胞组织细胞增生症共76例住院病人的资料总结分析。结果，76例病人67例依据皮疹印片或病理活检诊断(88．2％)，1例依据骨髓检查诊断，其余依据临床和依据临床和影像学检查诊断。皮疹印片18例，12例阳性；55例行病理检查，取材自皮肤、淋巴结、肿物(包括腹腔、头部、纵隔肿物)及骨损处穿刺或刮除物检查，找到郎格罕组织细胞；25例行S-100检查，24例阳性(96％)。39例行骨髓检查仅l例示组织细胞明显增加，其余示增生骨髓象或增生减低。结果表明：病理检查是郎格罕细胞组织细胞增生症的确诊的唯一手段。皮疹印片对LS病的诊断有特殊意义，骨髓检查诊断价值有限。HSC及EGB的病人诊断有赖于病灶局部肿物、淋巴结活检或病损骨组织的病理检查，S-100阳性有助于对不确定病例的诊断及提高诊断的可信度。     

以噬血细胞综合征为首发表现的儿童郎格罕细胞组织细胞增生症2例报告

郎格罕细胞组织细胞增生症(Langerhan’s cell histiocy-tosis,LCH)是一组病因不明,以郎格罕细胞为主的组织细胞在单核巨噬细胞系统广泛增殖、浸润为基本病理特征的疾病。此类细胞为良性,但生物学行为呈浸润生长,可累及骨与多个器官,好发于婴幼儿及儿童。此类疾病临床表     

郎格罕氏细胞组织细胞增生症诊断与鉴别诊断:附6例报告

郎格罕氏细胞组织细胞增生症(Langerhands cell histiocytosis，LCH)主要以郎格罕细胞浸润为特点。此细胞为良性，但生物学行为呈浸润生长，可累及骨及多个器官。好发于婴幼儿及儿童，发病率极低，多数临床医师缺乏对此病的认识。现对我院1988～2002年收治的6例LCH患儿病史进行分析。     

郎格罕细胞组织细胞增生症近期预后和器官功能衰竭

分析了３２例小儿郎格罕组织细胞增生症（ＬＣＨ）。近期预后为：＜６个月死亡１２例，发生脏器功能衰竭１２例。死亡８例，认为影响近期预后因素有：年龄（≤２岁）、合并脏器功能衰竭、骨髓增生低下或三系中受抑制，并探讨了ＬＣＨ的诊断学基础及治疗原则。     

Thymic Langerhans cell histiocytosis mimicking lymphoma

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by clonal expansion of antigen presenting Langerhans cells. Different clinical features can be seen according to the involved organs and systems. Multisystem disease with organ dysfunction is more common in infants, whereas single system disease is usually observed in older children. The disease can affect any system or organ throughout the body. Thymus is a rarely involvement site reported in LCH and usually is accompanied by skin, bone or lung disease. Here we report a 12-year-old male with thymic involvement by LCH clinically mimicking lymphoma.     

Growth of children with Langerhans cell histiocytosis

Diseases in childhood have an impact on growth. The influence of Langerhans cell histiocytosis (LCH) on growth has never been studied well. Recently a patient with LCH was treated with human growth hormone (GH) because of severe GH deficiency due to LCH involvement of both the hypothalamus and pituitary. This led us to review our charts from 1971 onward for evaluation of the growth patterns in patients with LCH. Here the long-term growth of 22 patients with LCH is reported, the median follow up being 7 years and 1 month. The height data were converted into standard deviation scores (SDS). At diagnosis the mean SDS of patients with isolated LCH at diagnosis was 0.04 and –0.37 in patients with disseminated LCH. Of the total group, 12 patients did not show any influence from the LCH or therapy on their growth. The remaining 10 patients reached, after a minimum of 3 years, a percentile clearly higher than that at diagnosis. However all the ten above mentioned patients, either isolated or disseminated LCH, had a lesion in the facial side of the skull.Conclusion GH deficiency is not a common manifestation of LCH in childhood and GH provocation tests are only indicated when there is a poor or decelerating growth rate. In our patients the number of organs involved and/or the treatment modality did not influence the growth in all but one.Both authors made equal contributions to this work and are listed in alphabetical order     

Pulmonary function testing and pulmonary Langerhans cell histiocytosis

In a long-term single-center follow-up (median 16-years), we studied high-resolution computed tomography (HRCT) and pulmonary function testing (PFT) in pulmonary LCH. Diffusing capacity corrected for alveolar volume (K(CO)) and total lung capacity (TLC) were significantly decreased (P=0.016 and P=0.030, respectively) in patients with extensive HRCT abnormalities. Patients with late stage disease on HRCT had increased forced expiratory volume (FEV1.0)(P=0.037) and vital capacity (VC)(P=0.036). Disease monitoring is important in pulmonary LCH, and since PFT with diffusing capacity provides a measurement of the current lung function, it may be a valuable tool in monitoring pulmonary LCH, and a good complement to imaging.     

Intralesional steroids in Langerhans cell histiocytosis of bone

Langerhans cell histiocytosis may involve single or multiple organ systems. Bone involvement is the most common feature. We have examined retrospectively the effects of 20 intralesional injections of steroids into bone in seven patients seen at our department from 1988 to 1993. Most of these injections (75%) relieved the symptoms, and no side-effects were observed. However, injections into the jaw were seldom effective. Our results suggest that the dose of the steroids administered is of importance.     

儿童朗格汉斯细胞组织细胞增生症的BRAF-V600E基因突变及临床意义

目的 探讨儿童朗格汉斯细胞组织细胞增生症（LCH）的BRAF-V600E基因突变的意义。方法 采用实时荧光定量PCR技术检测26例儿童LCH患儿石蜡包埋组织样本中的BRAF-V600E基因突变情况,并回顾性分析BRAF-V600E基因突变与临床特征及预后的关系。结果 25例患儿接受正规化疗,2年总生存率（OS）及无事件生存率（EFS）分别为100%、88%。70%（18/26）的病理标本来自骨组织,BRAF-V600E基因突变阳性率达50%（13/26）。BRAF-V600E基因突变与LCH患儿年龄、性别、受累器官、临床分类、早期治疗效果、复发情况以及2年OS及EFS均无相关性（P > 0.05）,但与LCH的临床分组相关（P < 0.05）。结论 LCH患儿总体生存率较高,BRAF-V600E基因突变发生率高,BRAF-V600E基因突变与LCH临床分组相关。     

Langerhans cell histiocytosis in children under 2 years of age

This is a retrospective study of 55 children under the age of 2 years diagnosed with Langerhans cell histiocytosis (LCH). They were classified according to age and organ function and dysfunction following Lahey's criteria. The studied population was divided into four groups by age of diagnosis (0–6, 7–12, 13–18, and 19–24 months). Statistical analysis showed no significant difference in outcome between age groups, although the population under 6 months had a 81.3% fatality rate. The presence of organ dysfunction was a major cause of death in all age groups, being statistically significant in outcome (P > 0.005) compared with patients without organ dysfunction. The presence of thrombocytopenia and/or respiratory dysfunction was also highly associated with a fatal outcome. In the surviving population, no second malignancies have been reported. The late secondary effects of therapy include endocrine, orofacial, and osseous pathologies. © 1996 Wiley-Liss, Inc.     

Langerhans cell histiocytosis with digestive tract involvement

Gastrointestinal tract (GIT) involvement in Langerhans cell histiocytosis (LCH) is not commonly described. We present two children presenting with GIT involvement with LCH, one successfully treated on standard protocol and other being treated on a protocol for relapsed disease. A review of literature showed almost 95% children were less than 2 years of age and 62% were females. Vomiting, abdominal pain, constipation, intractable diarrhea, malabsorption, bloody stools, protein‐losing enteropathy, and even intestinal perforation are some of the reported symptoms. More than 50% patients died within 18 months from diagnosis. Pediatr Blood Cancer. 2010;55:748–753. © 2010 Wiley‐Liss, Inc.