骨朗格汉斯细胞组织细胞增生症3例临床病理分析北大核心CSCD

目的探讨骨朗格汉斯细胞组织细胞增生症（langerhans cell histiocytosis,LCH）的临床病理特征、免疫表型、鉴别诊断及生物学行为。方法对3例骨LcH临床特征、组织学形态和免疫组织化学结果结合相关文献进行分析。结果2例男性,1例女性,发病年龄7～12岁（平均8．7岁）。病变部位分别位于肱骨、胫骨及额骨;镜下示Langerhans胞异常增生,伴有数量不等的嗜酸性粒细胞、淋巴细胞、中性白细胞及多核巨细胞;免疫组织化学提示,3例Langerhans细胞弥漫表达CD1a和s100。结论绝大多数发生于骨朗格汉斯细胞组织细胞增生症患者预后较好。其预后与病变部位、累及范围、有无器官功能异常及年龄相关。     

^18F-FDGPET／CT显像对朗格罕细胞组织细胞增生症的价值探讨

目的探讨^18F-FDGPET／CT显像在化疗前朗格罕细胞组织细胞增生症累及的器官及化疗后疗效的评价。方法14例1-14岁病理确诊为朗格罕细胞组织细胞增生症患儿均作PET／CT全身显像,其中3例是化疗前了解病变累及的器官;7例是化疗后评价疗效;4例为化疗前后对比,评价疗效。结果3例化疗前的患儿双侧颈部淋巴结肿大并摄取^18F-FDG增高,不同部位的骨质破坏并摄取^18F-FDG增高;7例化疗后的患儿有6例肿瘤活性已完全灭活,1例原病灶部位仍有高度活性的肿瘤组织存在;4例化疗前后对比发现,2例肿瘤活性完全灭活,1例原病灶摄取^18F-FDG减低,病情好转,1例病情加重,出现新发病灶。结论^18F-FDGPET／CT显像在朗格罕细胞组织细胞增生症化疗前了解累及器官的范围及化疗后评价疗效是一种较好的无创方法。     

朗格汉斯组织细胞增生症2例并文献复习

目的：探讨朗格汉斯组织细胞增生症的临床病理特点、鉴别诊断及生物学特征。方法：对2例朗格汉斯组织细胞增生症进行组织学观察和免疫组化染色，并复习相关文献。结果：两例患者分别为成人和儿童，均以头部肿物为首发症状，伴有不同程度的颅骨破坏。手术切除病灶后成人无复发，儿童一个月后颈部又发现肿物并侵犯横纹肌。2例均经病理学诊断为朗格汉斯组织细胞增生症。结论：朗格汉斯组织细胞增生症是一种影响网状内皮系统的疾病，主要以朗格汉斯细胞浸润为主，组织学为良性，但其生物学行为却呈侵袭性、破坏性，可累及骨、肺等多个器官。     

基于肺活检的原发性肺朗格汉斯细胞组织细胞增生症病理分型及预后分析

目的:总结肺朗格汉斯细胞组织细胞增生症(pulmonary Langerhans' cell histiocytosis,PLCH)临床病理特征及预后资料,提高对该病的诊断率,减少不良预后.方法:回顾性分析2002年至2019年间南京某医院经病理确诊的PLCH 7例,讨论其组织起源、临床特征、病理特点及预后.结果:7例...     

干扰素、白细胞介素2联合沙利度胺治疗成年人复发难治朗格汉斯细胞组织细胞增生症一例并文献复习

目的:探讨干扰素、白细胞介素2（IL-2）联合沙利度胺治疗朗格汉斯细胞组织细胞增生症（LCH）的效果及相关机制。方法:回顾性分析广西医科大学第一附属医院2012年6月收治的1例应用干扰素、IL-2联合沙利度胺治疗的复发难治LCH患者的临床资料，并复习相关文献。结果:应用干扰素、IL-2联合沙利度胺治疗后，患者临床症状好转，获得完全缓解，无明显药物不良反应。结论:干扰素、IL-2联合沙利度胺治疗复发难治LCH患者有效且不良反应小，可作为治疗复发难治LCH的一种新选择。     

宫颈朗格汉斯细胞组织细胞增生症的临床病理分析

目的探讨女性生殖道朗格汉斯细胞组织细胞增生症的临床病理学特征,以加深此病在宫颈等少见部位的认识。方法对1例宫颈LCH进行HE及免疫组化染色,观察其临床病理特征,并复习相关文献。结果患者女性,31岁,确诊为宫颈LCH,镜下朗格汉斯细胞弥漫增生,肿瘤细胞中等大小,细胞质淡粉染,核呈卵圆形,可见核沟,核仁不明显;背景可见较多嗜酸粒细胞及中性粒细胞等聚集,局部形成“嗜伊红微脓肿”,间质微血管及纤维母细胞增生明显。免疫组化:肿瘤细胞弥漫强表达CD1a和S-100,Langerin阴性,组织细胞CD68阳性。结论“纯”女性生殖道LCH极其少见,临床最常表现为瘙痒,可呈丘疹样体征,极易与湿疹、皮炎等皮肤病相混淆,临床及病理医师对女性生殖道LCH应保持警惕,当遇到难治性湿疹或宫颈糜烂等应考虑到是否存在该病可能。     

左腹股沟淋巴结内指突状树突细胞肉瘤1例报告并文献复习

目的: 探讨淋巴结内指突状树突细胞肉瘤的临床病理特征、诊断要点及鉴别诊断.方法: 对1例腹股沟淋巴结内指突状树突细胞肉瘤进行组织病理学和免疫组化染色观察并随访,结合文献进行讨论.结果: 镜下显示淋巴结结构破坏,细胞呈梭形、圆形、椭圆形,肿瘤细胞弥漫分布,异性型明显,可见核分裂象,经化疗后肿瘤内可见片状凝固性坏死.免疫表型:瘤细胞表达Vimentin、CD68和S-100蛋白.结论: 淋巴结内指突状树突状细胞肉瘤是一种罕见的免疫辅助细胞恶性肿瘤.其诊断依靠组织病理学和免疫组化染色,必要时电镜辅助诊断.应与滤泡树突细胞肉瘤、朗格汉斯细胞组织细胞增生症、恶性纤维组织细胞肉瘤、恶性黑色素瘤、间,变性梭形细胞癌及其他肉瘤鉴别.治疗以手术切除为主,辅以化疗和(或)放疗,预后差.     

骨骼朗格汉斯细胞增生症的治疗进展

朗格汉斯细胞增生症是以朗格汉斯细胞异常累积为特征的疾病,其发病原因尚不明确,发病率约为1∶1 500 000.患者以儿童为主,男性多见.根据朗格汉斯细胞增生症病灶累及部位及临床表现不同可分为嗜酸性肉芽肿,汗雪氏病（Hand-Schüller-Christian disease）及里斯氏病（Letterer-Siwe disease）.     

骨朗格汉斯细胞组织细胞增生症25例临床分析

 目的　分析骨朗格汉斯细胞组织细胞增生症（LCH）的临床特点,总结LCH诊断和治疗的方法。方法　回顾性分析2004年2月至2012年2月收治的25例经病理证实的LCH患者的临床资料及随访结果。结果　25例患者中男18例,女7例;中位年龄17岁。单发病灶17例,其中颅骨11例,多发病灶6例。首发症状多为疼痛和局部肿块,全身症状少见。主要症状多为局部疼痛,影像学表现为溶骨性改变,12例伴周围软组织肿胀。病理表现为分化好的组织细胞增生及大量嗜酸性粒细胞浸润,CD1a、S100、Vimentin、CD68免疫组织化学阳性率高。单发病例采用手术治疗为主,辅以放疗或化疗。多发病例以化疗为主,辅以放疗。仅累及骨的患者疗效满意,累及其他脏器的2例患者死亡。结论　骨LCH男性发病明显高于女性,好发于儿童和青少年,以单发病灶为多,颅骨侵犯多见。临床表现主要为局部疼痛和肿块,病理活组织检查是确诊的首选方法,治疗宜采用综合疗法。预后与骨病损范围和病理类型以及其他脏器受累情况,大部分病例预后好。     

朗格汉斯细胞肿瘤4例临床报道并文献复习

目的：探讨朗格汉斯细胞肿瘤临床表现、病理特点、治疗和预后。方法：描述一组成人朗格汉斯细胞肿瘤[包括3例朗格汉斯细胞增多症（LCH）和1例朗格汉斯细胞肉瘤（LCS）]患者的临床、病理表现、治疗情况等,并复习相关文献。结果：3例LCH患者分别为29岁女性、20岁男性以及35岁男性,临床受累脏器包括骨（2／3）、肝（2／3）、下丘脑-垂体（尿崩症）（2／3）、皮肤（1／3）,淋巴结（1／3）,甲状腺（1／3）、肺（1／3）。免疫组化为CDla（＋）、S-100（＋）,2例患者经4个疗程化疗,1例达到部分缓解,另1例无效。1例LCS患者为77岁女性,以颈部淋巴结肿大起病,淋巴结病理诊断为LCS,病情进展迅速,多脏器受累,死于多脏器功能衰竭。结论：成人LCH少见,临床表现不特异,容易误诊、漏诊,治疗需个体化。LCS临床罕见,侵袭性强,进展快,预后差。     

骨的Langerhans细胞组织细胞增生症8例临床病理分析

目的探讨骨的Langerhans细胞组织细胞增生症的临床病理学特征。方法对8例骨的Langer-hans细胞组织细胞增生症的临床资料和组织形态进行分析并采用免疫组织化学方法检测。年龄4～40岁,平均16岁。男5例,女3例。结果Langerhans细胞组织细胞增生症好发于青少年,组织学上由特征性的Langerhans细胞及嗜酸性粒细胞组成,免疫组织化学检测Langerhans细胞表达CD1a及S-100蛋白。结论Langerhans细胞组织细胞增生症是好发于骨的肿瘤性病变,发生在骨的Langerhans细胞组织细胞症要与慢性骨髓炎及恶性淋巴瘤相鉴别,该病治疗主要是手术刮除病灶及放疗,大多数患者预后良好。     

Histiocytose langerhansienne osseuse multifocale : place de la radiothérapie

Langerhans cell histiocytosis is a non-malignant proliferative disease of unknown etiology that can affect one or more organs. This is a rare disease, 1 to 2/100,000, affecting mainly children with a male predominance. The osseous lesions are the most frequent (60 to 90%). There is however no consensus treatment for the management of these sites. We report the cases of two patients successfully treated with radiotherapy after primary chemotherapy, at doses of 15 Gy in ten sessions of 1.5 Gy for one patient and 18 Gy in ten fractions of 1.8 Gy for the other. Single or multifocal bone Langerhans cell histiocytosis without visceral involvement is a benign, self-limiting affection in most cases. Some bone lesions could be treated by radiotherapy alone. But the high variability of doses currently given in the literature does not allow determining the lowest effective dose limiting the risk of secondary neoplasia or impaired growth in children, in whom lower doses of 6 to 8 Gy are recommended. The decision of radiotherapy must be weighed against the risk of the disease. Caution should be the rule in this non-malignant tumour pathology.     

Solitary Langerhans cell histiocytosis of frontal lobe: a case report and literature review

The brain parenchymal Langerhans cell histiocytosis （LCH） without systemic disease or lytic skull lesions is extremely rare. We report a 23-year-old male presenting with new onset 1 hour seizure with loss of consciousness 20 days prior to admission, and recurrent seizure 2 weeks later. Brain magnetic resonance imaging （MRI） showed an irregularly mass with enhancement involving the right frontal lobe. Microscopically, the lesion was characterized by sheets of Langerhans cells in addition to reactive inflammatory elements. Immunohistochemically, Langerhans cells were positive for Langerin, CDla and S-100 protein. The patient received no chemotherapy or radiotherapy after surgery. After 24 months of follow-up, no recurrence or other systemic lesions were observed. Although there is no standard treatment for solitary cerebral LCH, the prognosis generally appears to be good.     

Magnetic resonance imaging of thickened pituitary stalk proceeding to Langerhans cell histiocytosis in a child

Magnetic resonance imaging has shown isolated pituitary stalk thickening in certain cases of idiopathic or secondary central diabetes insipidus (DI) due to infiltrative processes. We present a 4‐year‐old boy who was initially diagnosed as having central DI. The MRI showed isolated pituitary stalk thickening with prominent homogeneous contrast enhancement. The remaining findings on MRI were within normal limits. The patient's personal and family history and laboratory and clinical findings were unremarkable; therefore, he was initially diagnosed as having idiopathic DI. Since central DI and isolated pituitary stalk thickening may be considered to be the first manifestations of Langerhans cell histiocytosis, we decided to follow up the patient. After 5 months, following the initial diagnosis, on skeletal X‐ray survey, the patient did indeed develop multiple lytic skull lesions which, on biopsy, were histologically typical bone lesions of Langerhans cell histiocytosis.     

Langerhans cell histiocytosis in endoscopic biopsy: marked pinching artifacts by endoscopy

Various diseases should be considered when evaluating a suprasellar mass. Diabetes insipidus has been reported as the primary manifestation in a patient with Langerhans cell histiocytosis involving the hypothalamopituitary axis (HPA) and can be helpful for the diagnosis. The use of endoscopic biopsy for suprasellar masses has increased. However, as it is difficult to make a diagnosis with endoscopic biopsy because of the many artifacts, pathologists must be careful. Langerhans cell histiocytosis involving the HPA as solitary lesions is rare. We report the eighth case that provides neurologists, radiologists, and pathologists with various implications.     

17例伴有肺部改变的朗格汉斯细胞组织细胞增生症的临床观察

目的 探讨儿童伴肺部改变的朗格汉斯细胞组织细胞增生症（PLCH）的临床表现、CT特征性表现、诊断及治疗特点,并分析其与预后的关系,为PLCH患者临床方案的制定提供依据。方法 回顾性分析本院2008年1月至2014年1月经病理确诊的17例PLCH患儿的临床特征、影像学资料及接受Ⅲ型化疗方案（诱导阶段6周+维持阶段52周）化疗后的疗效和预后。结果 17例PLCH患儿起病时伴有肺部弥漫性改变为主的多脏器损害,在疾病发展过程中,胸部CT表现出特征性变化。17例均接受Ⅲ型方案化疗,其中8例完全缓解,预后良好;4例化疗后复发,其中1例预后良好,3例死亡;3例化疗中疾病进展放弃治疗,死亡;1例化疗中疾病进展再诱导化疗,目前持续化疗中,存活;1例化疗维持治疗中,目前持续随访。结论 儿童伴肺部改变的LCH多为婴幼儿起病,伴多脏器损害,早期高强度化疗可改善预后,但部分病例肺部进展明显,预后不良。     

Disseminated Langerhans cell histocytosis in children: treatment outcome

Langerhans cell histiocytosis is an interesting disorder with a variety of presentations and variable outcomes. This study evaluates response to treatment, recurrence, and survival in disseminated Langerhans cell histiocytosis treated at Regional Cancer Centre, Trivandrum, India from 1983 through 1994. Thirty-five patients with disseminated Langerhans cell histiocytosis were seen. Six had group A disease, 21 had group B disease, and eight had group C disease. In group A, five of six patients are disease free at a median follow-up of 48 months. Two had recurrence after initial treatment, which was salvaged. In group B, 13 of 15 patients had complete response after chemotherapy, nine of whom experienced recurrence later. Three of five patients who received irradiation alone experienced recurrence. One died of progressive disease. Two patients were lost to follow-up. Seventeen of 20 are alive with median follow-up of 67 months. In group C, one of eight patients are alive after multiple recurrences. Of the surviving patients, 29% had significant sequelae. In summary, group A and B patients do well with treatment, and most of the recurrences can be salvaged. A significant proportion of patients have sequelae. Newer aggressive protocols must be developed for treating group C patients. Measures to prevent sequelae must also be developed.     

Histiocytosis Syndromes Classification, Diagnostic Features and Current Concepts

The histiocytosis syndromes are broadly classified as those that feature dendritic cells and those that display only ordinary histiocytes in pathological lesions. The leukemias and lymphomas are malignant but the others defy simple assignment to basic pathological categorization. The ontogeny of histiocytes is considered, Langerhans cell histiocytosis and the hemophagocytic syndromes are discussed in detail and a conceptual context is presented     

Functional imaging of Langerhans cell histiocytosis by (111)In-DTPA-D-Phe(1)-octreotide scintigraphy

BACKGROUND: Langerhans cell histiocytosis is a rare group of diseases of unknown pathogenesis, variable clinical presentation, and behavior. It encompasses highly aggressive, often fatal, and indolent diseases, with a variety of intermediate forms between the two extremes. Localization and monitoring of Langerhans cell histiocytosis deposits are based on the combined use of different imaging modalities, which include magnetic resonance, computed tomography, and nuclear medicine procedures. Somatostatin receptor scintigraphy recently has been used to image immune-mediated diseases with excellent results. METHODS: In this study, the authors have evaluated the potential role of somatostatin receptor scintigraphy by using (111)In-DTPA-D-Phe(1)-octreotide in a series of patients with Langerhans cell histiocytosis. Eight consecutive patients (2 males, 6 females; mean age, 43 years; age range, 29-60 years) with histologically proven Langerhans cell histiocytosis, in various phases of their disease, were imaged 24 hours after intravenous injection of 111-222 MBq of (111)In-DTPA-D-Phe(1)-octreotide, by using total body and spot view images. The objective of the study was to preliminarily evaluate this technique in the evaluation of Langerhans cell histiocytosis. RESULTS: Most Langerhans cell histiocytosis deposits were clearly documented by somatostatin receptor scintigraphy, independently from the anatomic location (i.e., skeleton, mucosae, soft tissue, etc.). The correlation between conventional imaging modalities and somatostatin receptor scintigraphy results was fairly good, though this was not considered a primary objective of the study. CONCLUSIONS: Among the various imaging modalities for Langerhans cell histiocytosis, somatostatin receptor scintigraphy is unique, because it accurately depicts the active sites of disease and thus can help in monitoring the response to treatment, together with conventional imaging modalities. Further studies are necessary to confirm the diagnostic value of this technique and clarify the biologic significance of the expression of somatostatin receptors in Langerhans cell histiocytosis.