肺朗格汉斯细胞组织细胞增生症1例

朗格汉斯细胞肉芽肿病是指朗格汉斯组织细胞增生性病变,以前包括在组织细胞增生症X中。最近研究表明朗格汉斯细胞肉芽肿病的主要病变是朗格汉斯细胞的克隆性增生。目前认为朗格汉斯组织细胞增生症更合适。肺朗格汉斯细胞组织细胞增生症（pulmonary Langerhans cell histocytosis,PLCH）为朗格汉斯组织细胞增生症的一种类型,可以原发肺部,也可是全身系统病变的一部分。     

3例朗格汉斯细胞组织细胞增生症随访报告

朗格汉斯细胞组织细胞增生症（Langerhanscellhistiocytosis,LCH）,是一组临床表现各异的综合征的总称,包括Hand．Schuller．Christian综合征（韩．薛．柯病,侵犯全身多个器官、慢性起病,具有骨质缺损、突眼和尿崩症三联症者）、Letterer—Siwi病（勒．雪病,侵犯全身多个器官,急性起病者）、嗜酸性肉芽肿（eosinophilicgranuloma）,局限于肺的孤立型病变,病情相对缓和者。其发病、临床症状及病变范围差异很大。1995年至今,我们所在科室曾诊治4例,其中1例的有关资料已另有报道。     

成人脊柱朗格汉斯细胞组织细胞增生症误诊1例

脊柱朗格汉斯细胞组织细胞增生症发病率低,临床表现无特异性,且病理组织获得难度大,因此临床误诊及漏诊率高.本文对1例开始误诊为脊柱结核最终通过手术病理活检确诊为脊柱朗格汉斯细胞组织细胞增生症的病例进行报道,以提高对本病的认识,减少误诊率.     

肺朗格汉斯细胞组织细胞增生症1例并文献复习

临床资料 患者,男性,46岁,装修工人,长期接触粉尘10余年。吸烟20余年,平均20支／d。主要症状为活动后呼吸困难,咳嗽,咯白色粘痰,偶伴有胸痛,无咯血、畏寒、发热、盗汗等不适。患者患病以来,精神食欲欠佳,大小便无明显异常,睡眠尚可,     

吸烟与肺朗格汉斯细胞组织细胞增生症

肺朗格汉斯细胞组织细胞增生症的发生与吸烟有关.烟草糖蛋白可引起一系列细胞和细胞因子病理变化.戒烟可使部分患者病情稳定,改善影像学变化,甚至可使疾病(包括肺外病变)痊愈.     

小儿朗格汉斯细胞组织细胞增生症9例临床分析

目的提高小儿朗格汉斯细胞组织细胞增生症（LCH）的诊断与鉴别诊断水平。方法回顾性分析9例LCH患儿的临床资料,总结其症状、影像学表现及治疗。结果 9例患儿均以头部一过性软组织包块就诊,其中伴局部疼痛2例,突眼1例,咳嗽1例,乏力及低热3例,多尿1例;CT表现为局限性或多发性溶骨性骨质缺损,边界清晰,周围无破坏,伴有软组织肿块,MR示板障破坏累及颅骨内外板,伴软组织肿块,病变部位呈长T1长T2信号,增强后强化明显。9例经病理证实为LCH,术后3例辅以泼尼松＋长春新碱＋依托泊苷化疗6周,2例行局部放疗（36 GY/20 F）,随访3个月～10年,死亡1例,余8例预后良好,无复发。结论 LCH确诊需结合临床表现、影像学检查和病理活检;对以一过性头部软组织包块为首诊的小儿患者,要考虑到LCH的可能。     

单独肺受累的成人肺朗格汉斯细胞组织细胞增生症1例

1临床资料 患者,男,46岁,于2010年4月11日在绍兴县中心医院健康体检发现右下肺占位,胸部CT示右下肺占位,考虑炎性假瘤,建议治疗后复查,右上肺多发炎性病变。既往体健,无结核等特殊病史,吸烟20年,25支／d,近3个月来偶有右侧后背部隐痛不适,无咳嗽、咳痰,无畏寒、发热等呼吸系统疾病症状,于当地医院输液抗炎治疗3d（具体不详）,为求进一步诊治于2010年4月16日就诊于绍兴市人民医院。     

成人肺朗格汉斯细胞组织细胞增多症五例临床分析

目的 探讨成人肺朗格汉斯细胞组织细胞增多症(PLCH)患者的临床、影像、肺功能和组织病理特点.方法 回顾性分析2006年6月至2007年10月北京朝阳医院收治的5例PLCH患者的临床资料.结果 5例均为男性吸烟者,以肺部损害为主,其中2例以自发性气胸为首发症状,1例为肺脏终末期病变合并肺动脉高压.胸部高分辨率CT示大小不一的多发囊腔阴影,病变以双上肺为著,终末期病例囊腔影遍及全肺.5例均经外科肺活检获得组织病理学资料,显示囊腔样改变和朗格汉斯细胞聚集(免疫组织化学CD1a和S-100染色阳性).肺功能检查示V50占预计值%为53.6%～77.6%,V25占预计值%为38.5%～70.5%,V50和V25的降低与组织病理学所见小气道受累相符合.治疗以戒烟为主,疾病有自然缓解倾向.结论 PLCH是朗格汉斯细胞组织细胞增多症的一种特殊类型,主要发生于吸烟者,多呈良性经过.     

婴儿朗格汉斯细胞组织细胞增生症侵犯十二指肠一例

朗格汉斯细胞组织细胞增生症多见于儿童,男性多见。南京医科大学附属儿童医院近期收治了1例反复红色皮疹并渐进增多伴发热患儿,皮疹压片、十二指肠活检发现朗格汉斯细胞,免疫组化提示Langerin、CD1α、S-100蛋白阳性,结合患儿既往存在皮肤、骨损伤,病程中出现反复呕吐,胃镜及活检提示十二指肠受累,最终诊断为朗格汉斯细胞组织细胞增生症（多灶、多系统型勒雪病）,考虑到多系统受累及预后差,转入血液科行长春花碱联合泼尼松治疗,随访15个月皮疹消退,症状改善,病情稳定。     

肺朗格汉斯细胞组织细胞增多症合并肺动脉高压

目的 探讨肺朗格汉斯细胞组织细胞增多症(PLCH)合并肺动脉高压(PH)的临床表现,以提高对本病的认识.方法 回顾性分析2006年6月至2011年6月首都医科大学附属北京朝阳医院呼吸与危重症医学科收治的11例PLCH患者的临床资料.结果 11例PLCH患者中4例合并PH(36％),PLCH-PH临床症状较无PH者重,表现为Borg呼吸困难评分明显增加,杵状指,心功能(NYHA)达Ⅲ～Ⅳ级,并出现右心衰竭体征.胸部HRCT以双上中肺野弥漫囊腔样损害为主,并可见肺动脉增宽,右心增大.肺一氧化碳弥散量及动脉血氧分压也显著降低,并出现呼吸衰竭.彩色多普勒超声心动图示肺动脉收缩压升高.肺活检病理除朗格汉斯细胞浸润及囊腔样改变外,还可见肺小血管管腔狭窄及毛细血管扩张.治疗以氧疗、对症缓解症状为主,3例接受激素或联合免疫抑制剂治疗未见明显效果;4例PLCH-PH,仅1例随访1年病情稳定.结论 PH是PLCH比较常见的并发症,是PLCH病情进展、预后不良的标志;对于PLCH患者应注意PH的早期评估与预防.     

郎格罕组织细胞增生症五例临床分析

目的 提高对郎格罕组织细胞增生症（LCH）的认识。方法 回顾分析我院近10年来确诊的5例LCH成年患者的临床、影像和病理资料。结果 男3例,女2例,平均年龄36．8岁。所有病例均表现有肺、骨骼、中枢神经系统、皮肤、肝、脾、淋巴结等多器官病变。LCH肺部病变的典型放射影像学表现为双肺弥漫结节影,间质纤维化伴多发囊疱形成。有3例患者行骨X线检查,可低密度骨质破坏灶。全部患者的活检标本均可见异常的郎格汉斯细胞浸润。对5例患者均予反复全身化疗（激素＋蒽环类细胞毒药物）,化疗对肝、脾、淋巴结、皮肤病变的疗效较好,而对肺部病变、中枢性尿崩症、骨损害的疗效较差。结论 LCH可在任何年龄发病,对有尿崩症、特征性骨质破坏和肺部病变的患者应警惕此病,并及时行病灶部位的病理学检查,确诊后予放疗、全身化疗治疗。     

朗格汉斯细胞组织细胞增生症二例临床分析

目的 探讨朗格汉斯细胞组织细胞增生症（LCH）的临床特征.方法 回顾分析2例LCH患者的临床资料并复习相关文献.结果 2例均为未成年男性患儿颅骨受累伴骨质破坏,1例为单发灶累及眶后壁导致突眼,另1例为多发灶导致额部及枕部多发包块,2例均经病理学证实为LCH（其中1例为嗜酸性肉芽肿）.第1例给予病灶切除术加放疗,第2例未行放化疗.结论 LCH是一种综合征,头颈部受累很常见,病灶定位于颅骨时可表现为头部包块、眼球突出等,临床上出现上述症状时应考虑LCH可能.确诊需要病理学及免疫组织化学证据.治疗根据临床表现及受累器官选择治疗方案.预后与受累器官数目和功能受损情况密切相关.     

Cytogenetic abnormalities in PHA-stimulated lymphocytes from patients with Langerhans cell histocytosis. AIEOP-Istiocitosi Group

The aetiopathogenesis of Langerhans cell histiocytosis (LCH) is still undefined. Constitutional abnormalities in LCH have rarely been reported. One study showed chromosomal instability in lesional cells from three patients. No chromosomal studies are available on peripheral blood lymphocytes. Peripheral blood lymphocytes were analysed for the presence of chromatid and/or chromosomal breaks and structural rearrangements. A fluorescence in situ hybridization (FISH) painting technique was also applied in two cases. Sixteen patients with multisystem (MS, n = 11) or single system (SS, n = 5) LCH were studied, either at the diagnosis (n = 8), during treatment (n = 2) or during follow‐up, when asymptomatic (n = 6). Thirteen patients had chromosomal abnormalities. Eleven patients (69%) had chromatid and chromosomal breaks in 7–45% of cells. Overall, chromosome and chromatid breaks were significantly more frequent in the 11 patients with MS disease than in the five patients with SS disease: the mean percentage of cells showing chromosome and chromatid breaks was 13·4% in MS patients vs. 6·2% in SS patients (P = 0·003). Chromosomal abnormalities may be found in phytohaemagglutinin (PHA)‐stimulated peripheral blood lymphocytes of LCH patients at diagnosis, during the disease course and even during long‐term follow‐up, more frequently in MS disease. Chromosome instability may be considered as either a basic genetic instability or as a landmark of reaction to an environmental agent (viral?) that, through genome alteration, may play a role in histiocyte proliferation and, in some cases, also in the increased risk of malignancy.     

肺郎格罕细胞组织细胞增多症7例临床分析

目的探讨肺郎格罕(Langerhans)细胞组织细胞增多症的临床表现,以提高对本病的认识。方法回顾性分析1997—2006年北京协和医院确诊的7例肺郎格罕细胞组织细胞增多症的临床资料。结果7例患者均为男性,平均年龄26.7岁,其中4例吸烟,主要症状为咳嗽、活动后气短,5例在疾病过程中发生气胸。肺功能示阻塞性通气功能障碍3例、限制性通气功能障碍2例、混合性通气功能障碍2例,4例有不同程度的弥散功能障碍。胸部高分辨CT(HRCT)示6例表现为双上中肺野网格状改变及囊性变,仅有1例可见小结节影。外科肺活检标本病理学检查结果示7例光镜下均可见病理性郎格罕细胞浸润,6例可见囊样及气腔样结构。免疫组化阳性检出情况为7例S-100均为阳性;5例行CD1a检查者中4例阳性;6例行CD68检查者中5例阳性。结论肺郎格罕细胞组织细胞增多症常见于年轻吸烟男性,主要临床表现为咳嗽、活动后气短、反复气胸,胸部HTCT表现为双上中肺野为主的网结节或囊性变,肺功能无特异性改变,可伴有弥散功能异常,病理学检查可见病理性郎格罕细胞或免疫组化CD1a、S-100阳性可以明确诊断。     

ESHAP therapy effective in a patient with Langerhans cell sarcoma

We describe the rare case of a 53-year-old woman with systemic involvement of Langerhans cell sarcoma (LCS) who had undergone living-related liver transplantation. We chose the CHOP regimen as first-line chemotherapy, and clinical improvement of LCS was obtained. Intensive care was necessary due to the systemic involvement of LCS and severe infectious diseases. After the third cycle of CHOP therapy, however, disease progression was observed, and we administrated a modified ESHAP regimen (etoposide, carboplatin, cytarabine, methylprednisolone) as second-line therapy. A marked response was obtained after four cycles of this combination chemotherapy. Modified ESHAP may be a very effective combination chemotherapy regimen for LCS.     

吸烟相关性肺朗格汉斯细胞组织细胞增生症病例报告和文献复习

目的 观察吸烟相关性肺朗格汉斯细胞组织细胞增生症(pulmonary Langerhans cell histiocytosis,PLCH)病理学、免疫组织化学特征及其影像学的动态变化.并复习有关PLCH的诊治进展.方法 1例经开胸肺组织活检、免疫组织化学和高分辨率CT(HRCT)证实的吸烟相关性PLCH患者,动态观察戒烟1年后肺HRCT影像学改变.结果 患者有吸烟史(吸烟指数200),确诊后经戒烟1年后肺HRCT显示结节性和囊性病变完全消失.结论 吸烟是本例PLCH患者发病主要原因,戒烟是治疗吸烟相关性PLCH的主要方法.     

Adult Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) is a proliferative histiocytic disorder of unknown cause originating from dendritic cells. The clinical presentation of LCH is highly variable. Although the features of this disease have been well described in children, they remain poorly defined in adults. Here, we review the current knowledge about adult LCH, focussing on clinical presentation, diagnosis, treatment, and prognosis.     

Telomere length shortening in Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) is a clonal, proliferative disorder of phenotypically immature CD1a⁺ Langerhans cells (LC). The aetiology of LCH is unknown and data supporting an immune dysregulatory disorder as well as a clonal neoplasm have been reported. Telomere shortening has been associated with cancers and premalignant lesions as well as promoting chromosomal instability. To determine whether LCH LC have altered telomere lengths, we used dual detection of CD1a expression by immunofluorescence and telomere length by fluorescence in situ hybridization of LCH LC and lymphocytes in local, multisystem and systemic LCH and compared these with telomere lengths of LC and lymphocytes in reactive lymph nodes. LCH LC showed significantly shorter telomere lengths than LC from reactive lymph nodes or unaffected skin. Lymphocyte telomere lengths showed similar profiles among the different samples. These data show a significant telomere shortening in LCH LC in all stages of disease involvement compared with LC from reactive lymph nodes, suggesting that LCH may share mechanisms of telomere shortening and survival with clonal preneoplastic disorders and cancer, although an initiating infectious or immune event is still possible.