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.     

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是一种较为罕见的疾病.由于朗格汉斯细胞异常增生、浸润导致肺部结节、囊样改变.最终导致肺纤维化.本病主要累及20～40岁的吸烟人群,病因不明,亦无特异性治疗.本文对近来关于该病的报道作一综述.     

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。     

肺郎格汉斯细胞组织细胞增多症一例

患者男性，33岁，因常规体检时发现两肺弥漫性病变于2006年6月25日入院。患者于3个月前感冒后出现轻微咳嗽，无痰、咳血，无发热、喘息及夜间盗汗而未进行治疗。1周前参加常规健康体检，X线胸片发现两肺弥漫性病变于2006年6月15日曾在当地医院就诊。当天做胸部CT检查考虑为支气管扩张。患者既往身体健康，     

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.     

肺朗格汉斯组织细胞增多症

朗格汉斯组织细胞增多症(langerhans cell histiocytosis，LCH)是一组疾病的总称，以过量活化的朗格汉斯细胞(langerhans cell，LC)在器官内增殖、浸润为特征性标志，可有多系统受累，包括肺、骨、皮肤、前列腺、肝脏、淋巴结和胸腺等。根据器官受累范围，LCH被划分为单器官受累(如肺朗格汉斯组织细胞增多症pulmonary langerhans cell histiocytosis，PLCH)、多器官受累(如Hand—Schueller-Christian)、多系统受累(如Letterer—Siwe)。其中，PLCH是LCH的一个组成部分，这种命名法反映了该病的细胞学基础，以及相对独立的肺脏受累。有资料显示，大约4％～20％的PLCH患有囊性骨病变；此外，纵隔淋巴结、前列腺、皮肤、心脏、脑、肠等也可受累。这提示：PLCH可单独累及肺脏，也可侵犯其他脏器。     

累及垂体和甲状腺的成人朗格汉斯细胞组织细胞增生症1例

朗格汉斯细胞组织细胞增生症（Langerhans cell histiocytosis,LCH）是以CD1a +/CD207 +髓样树突状细胞克隆增殖为特征的组织细胞性疾病。根据受累部位和范围的不同,LCH的临床表现及治疗差异均较大。本文对1例累及垂体和甲状腺的成人LCH进行临床特点分析,以探索...     

肝朗格汉斯组织细胞增多症1例

1病例介绍 患者男性,23岁,因＂间断乏力3年,加重伴尿黄2月余＂于2010年7月1日入院。患者3年前无明显诱因出现乏力、消瘦,未予诊治。1年前体检发现肺部阴影、肝功能异常（具体数值不详）,于外院诊为＂继发性肺结核＂,予抗结核药物治疗8个月,后因疗效不佳停药。2月前,患者自觉乏力症状加重伴尿黄,于多家医院诊疗,查肝功能显著异常,     

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。     

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。     

Pulmonary histiocytosis X

Pulmonary histiocytosis X is a granulomatous disorder of the lung of unknown cause. Patients with this disease often complain of cough, dyspnea on exertion, and, occasionally, chest pain from a pneumothorax or bone involvement. However, it is not unusual for patients to be asymptomatic with only a diffuse interstitial infiltrate on their chest radiograph. The course is unpredictable and can range from spontaneous remission to progressive respiratory insufficiency and death. No therapy, beyond symptomatic and supportive care, has been shown to be effective. Pulmonary histiocytosis X appears to be a variation of the more disseminated histiocytosis X disorders, such as Letterer-Siwe disease and the Hand-Schüller-Christian syndrome. All of the histiocytosis X disorders have a characteristic histiocyte on pathologic study, with a unique ultrastructural organelle. Recent work suggests that this histiocyte is similar to macrophages found in the skin and some lymphoid organs. The histiocytosis X disorders may represent an abnormal response of the mononuclear phagocyte system to an antigen, to an immunodeficiency state, or both.     

Complete disappearance of lung abnormalities on high-resolution computed tomography: A case of histiocytosis X

A case of pulmonary Langerhans cell histiocytosis, proved by both lung high-resolution computed tomography and lung biopsy, is described. Following smoking cessation, lung nodules and cysts gradually disappeared on serial computed tomography scans, with complete clearance of the lesions after 12 months. The role of tobacco smoking is discussed, in detail, against the background of the literature.     

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.     

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.     

Sclerosing cholangitis as a complication of Langerhans'cell histiocytosis

INTRODUCTION: Langerhans'cell histiocytosis is a rare and mainly pediatric disease. Patients with hepatic involvement usually have a disseminated form of the disease, with poor prognosis. Sclerosing cholangitis can occur in 10 to 15% of multivisceral Langerhans'cell histiocytosis. We report the case of a 56-years-old patient who developed sclerosing cholangitis 12 years after the diagnosis of Langerhans'cell histiocytosis. EXEGESIS: A 56-years-old man was admitted because of rapid general health impairment with epigastric pain. He was diagnosed as having Langerhans'cell histiocytosis 12 years ago because of a diabetes insipidus. Lungs were involved and during follow-up vertebral osteocondensation also developed. However, Langerhans'cell histiocytosis was clinically silent at the time of admission, without any treatment. Biologically, cholestasis and inflammation were noted. Digestive radiological investigations (echo-endoscopy, CT-scan, MRI) showed homogenous hepatomegaly and a diffuse infiltration of intra and extrahepatic bile ducts. Liver biopsy yielded the diagnosis of sclerosing cholangitis. Clinical and biological improvement occurred with oral corticosteroids (at 12 months after sclerosing cholangitis diagnosis). CONCLUSION: Sclerosing cholangitis is a potential complication of Langerhans'cell histiocytosis, mainly in its multivisceral form. It can occur at a median of 2 years after diagnosis in children, but occasionally much later in adults, whereas Langerhans'cell histiocytosis seems quiescent. Diagnosis is supported by radiological investigations and liver biopsy. As no drug therapy appears clearly effective, liver transplantation must frequently be considered in these patients.     

Value of CD-1-positive cells in bronchoalveolar lavage fluid for the diagnosis of pulmonary histiocytosis X

Pulmonary histiocytosis X is characterized by an accumulation of CD-1-positive histiocytosis X cells in the lung, which also can be found in the bronchoalveolar lavage fluid (BALF). However, it has recently been demonstrated that CD-1-positive cells can also be detected in BALF of patients with other interstitial lung diseases and in healthy smokers. We therefore examined the frequency of CD-1-positive cells in a pool of patients with different pulmonary disorders, according to their smoking habits and diagnoses. We have studied the bronchoalveolar lavage in patients with pulmonary histiocytosis X (n=6), sarcoidosis (n=88), and in 97 patients with other miscellaneous lung disorders by using the immunoperoxidase method to detect CD-1-positive cells on glass slides. All patients with histologically proven histiocytosis X displayed more than 5% CD-1-positive cells, whereas patients with other pulmonary disorders showed no more than 3.6% CD-1-positive BAL cells. The dividing line of 5% CD-1-positive cells was not influenced by patients’ smoking habits. The identification of CD-1-positive cells in BALF appears to be useful in diagnosing pulmonary histiocytosis X.     

Clinical features of five cases of pulmonary Langerhans' cell histiocytosis]

We reviewed the clinical features of 5 cases of Langerhans' cell histiocytosis that had been referred because of pulmonary lesions. The most frequent symptom was persistent dry cough. Chest radiographs showed bilateral, symmetric reticulonodular infiltrates and accompanying cystic changes with an upper-field predominance. Pulmonary function testing (PFT) revealed moderate restrictive impairment in 3 patients. All the above features were in accordance with previous reports. The incidence of complications was, however, higher in the present cases than reported in the literature. Four cases were complicated with diabetes insipidus (DI), which caused polydipsia and polyuria on the initial presentation and was subsequently managed with intranasal desmopressin. In 4 cases, bone lesions were detected. A bone scintigram at 99mTc proved to be useful for surveillance. All patients had been followed up closely for longer than 2 years (median duration 2.8 years). Their clinical courses were generally stable without apparent decline in PFT, except that one patient with a psychiatric problem died from hypernatremia due to misuse of desmopressin. Immunosuppressive agents were given in only 2 patients including the one who died. Four transbronchial biopsies (TBB) were performed in 4 cases and at least 3 specimens were sampled from each. Histological diagnoses were made from TBB specimens in 3 patients, while the remaining 2 patients underwent an open lung biopsy. We conclude that TBB is acceptable as an initial diagnostic attempt. Multiple sampling may also contribute to preferable yield. Regarding patient management, we suggest that DI and bone lesions should be sought extensively as their incidence may be much higher than previously estimated.     

Clinico-epidemiological features of pulmonary histiocytosis X.

OBJECTIVE: To define the clinico-epidemiological features of pulmonary histiocytosis X in Japan. METHODS: A nationwide survey was carried out in 1997 using two questionnaires. RESULTS: The first questionnaire, which attempted to determine the number of patients during 1996, revealed that the number of patients treated at hospitals with 200 or more beds during the one-year period was estimated to be 160 (95% confidence interval: 140-180). The estimated crude prevalence among those aged 16 to 70 years was calculated as 0.27 and 0.07 per 100,000 population in males and females, respectively. The second questionnaire was concerned with the clinico-epidemiological features of the disease. Seventy-three histologically diagnosed patients were evaluated. It primarily afflicted younger adults, between the ages of 20 and 50, and showed a male predominance. Over 90% of the patients were smokers or ex-smokers and over 50% started smoking before 20 years of age, suggesting a strong association with cigarette smoking. Steroid therapy was applicable to 34% of the patients. In the patients who received steroid therapy, regression and stabilization were observed in 28% and deterioration in 36%. As for the patients for whom steroids were not required, remission occurred in 63% and progression in 10%. The ratio of remissions plus stabilization was higher in the patients who were not treated with steroids compared with those who required steroid therapy (p<0.05). CONCLUSION: In patients with pulmonary histiocytosis X therapeutic results obtained with steroids seemed not to be encouraging, although steroids are thought to be the most plausible treatment.     

累及气道的窦组织细胞增生症伴巨大淋巴结病一例并文献复习

目的 提高对累及气道的窦组织细胞增生症伴巨大淋巴结病(Rosai-Dorfman病)的认识.方法 分析1例确诊为气道受累的Rosai-Dorfman病患者的临床表现、支气管镜下表现、病理特征、诊断和治疗并结合文献进行讨论.结果 患者女,60岁.因"反复发作性喘息18个月,加重1个月"于2007年3月6日收入福建省立医院.曾诊断为"支气管哮喘",口服强的松、吸入布地奈德等治疗,症状好转.1个月前喘息再次发作,且出现吸气性呼吸困难,平卧位时明显,再次就诊,以"呼吸困难原因待查"收入院.23年前发现"高血压、左肾上腺腺瘤",诊断为"原发性醛固酮增多症",未行手术.1999年发现左腮腺肿物、左颌下淋巴结肿大,行手术切除.2000年行双眼泪腺肿物切除.2001年发现全身皮下多发性结节,且逐年增多,结节可自行增大和缩小.入院检查左颌下可触及大小约3.0 cm×2.0 cm结节,双上臂、胸背部、腹部、臀部及双大腿外侧可扪及黄豆至蚕豆大小结节,边界清楚,质偏硬,表面尚光滑,稍活动,轻压痛,部分皮肤见色素沉着.胸部CT平扫+气管重建显示气管壁多发性结节状突起,相应区域管腔狭窄,左肺舌叶炎症,纵隔内未见明显肿大的淋巴结,胸背部皮下多发性结节影.淋巴结病理切片可见组织细胞形成的浅染区与浆细胞和淋巴细胞形成的深染区相间排列,呈一种窦样结构,组织细胞胞质内可见吞噬的浆细胞和淋巴细胞,确诊为Rosai-Dorfman病.结论 Rosai-Dorfman病累及气道是罕见的淋巴结外病变,易发生误诊和漏诊.支气管镜检查是发现Rosai-Dorfman病气道受累的重要手段,组织细胞增生并吞噬完整的浆细胞和淋巴细胞是其病理特征,免疫组织化学染色S100蛋白及CD_(68)阳性有助于诊断和鉴别诊断.手术切除联合应用糖皮质激素或放疗是治疗气道病变较为有效的方法之一.