雷公藤左旋咪唑混悬液对表皮内Langerhans'cells的影响

目的探讨雷公藤左旋咪唑混悬液对皮肤表皮内Ｌａｎｇｅｒｈａｎｓ＇ｃｅｌ（ＬＣ）的影响。方法室温下经雷公藤左旋咪唑混悬液浸泡人和大鼠皮片３ｈ，采用ＡＴＰ酶法显示表皮片中的ＬＣ。结果显微镜下见：ＬＣ数量减少，胞体浓缩或崩溃变形，树突状结构明显减少。ＬＣ数减少与生理盐水对照有显著性差异。结论雷公藤左旋咪唑混悬液体外浸泡皮片对表皮内ＬＣ的数量和结构均有影响，从而使ＬＣ功能受抑，同种异体皮肤移植排斥延迟。

Langerhans cell histiocytosis misdiagnosed as thyroid malignancy: A case report

BACKGROUND The incidence of Langerhans cell histiocytosis(LCH) is low, and involvement of the thyroid is even rarer, which results in high missed diagnosis or misdiagnosis rates.CASE SUMMARY We report a young woman with a thyroid nodule. Thyroid malignancy was suggested by fine needle aspiration, but she was eventually diagnosed with multisystem LCH, thus avoiding thyroidectomy.CONCLUSION The clinical manifestations of LCH involving the thyroid are atypical, and the diagnosis depends on pathology. Surgery is the main method for treating primary thyroid LCH, while chemotherapy is the main treatment method for multisystem LCH.

Adult localized Langerhans cell histiocytosis:A case report

BACKGROUND Langerhans cell histiocytosis(LCH)is a rare clonal proliferative disease of Langerhans cells with unknown pathogenesis.An increasing number of clinicians recognize that LCH has a wide clinical spectrum and a highly varied course.Adults rarely develop LCH.Here,we report a case of adult localized LCH.CASE SUMMARY A 32-year-old woman presented with plaques and ulcers on the vulva and crissum,accompanied by pain that persisted for more than one year.Physical examination revealed a red-infiltrating plaque with ulcerations and exudates in the vulva and crissum.Pathological examination revealed a diffuse infiltration of lymphocytes,eosinophilic granulocytes,and histiocytoid cells in the superficial dermis.Proliferative histiocytoid cells showed mild atypia,partly with kidneyshaped nuclei.Immunohistochemical examination showed that the histiocytoid cells were positive for S100 protein and CD1 and weakly positive for CD68(20%+),with a Ki-67 index of 30%.Laboratory tests did not reveal any other systemic damage.The patient was diagnosed with adult localized LCH and was prescribed oral prednisone(20 mg)once daily.The skin lesions gradually improved and are still being followed-up.CONCLUSION Adult localized LCH is rare and must be differentiated from other common conditions.

Targeting epidermal Langerhans cells by epidermal powder immunization

Immune reactions to foreign or self-antigens lead to protective immunity and, sometimes, immune disorders such as allergies and autoimmune diseases. Antigen presenting cells (APC) including epidermal Langerhans cells (LCs) play an important role in the course and outcome of the immune reactions. Epidermal powder immunization (EPI) is a technology that offers a tool to manipulate the LCs and the potential to harness the immune reactions towards prevention and treatment of infectious diseases and immune disorders.

ASSAY OF S100+ LANGERHANS' CELLS IN CERVICAL CARCINOMA

Langerhans cells (LCs) in the normal cervix (control, 19 cases), cervical carcinoma in situ( CIS, 19 cases) and invasive cervical carcinoma (30 cases), interdigitatlng cells (IDCs) and follicular dendritic cells (FDCs) in obturator lymph node draining Invasive cervical carcinoma were quantitatively assessed by ABC immunohistochemical staining using anti-S100 protein antibody. The results indicated that S100+ LCs in sltu, S100+ IDCs and S100+ FDCs In obturator lymph node showed dendritic features with a specific distribution. Number of LCs in situ in Invasive carcinoma increased significantly when compared with CIS and control groups. There was no change in LCs number between grade Ⅱ and Ⅲ of squemous cell carcinoma. Number of IDCs was significantly less in stage Ⅱ than in stage Ⅰ . FDCs number In different Invasive depthes and clinical stages showed no obvious change. The results suggest that progression of cervical carcinoma is closely related to decrease of LCs hi situ and IDCs in regional lymph node. Predominant Immune response of the host to cervical carcinoma should be cellular immunity.

Hupo powder promotes autophagy of menstrual blood-derived stem cells from patients with endometriosis

Objective:To explore the effect of Hupo powder(HP)on autophagy in menstrual blood-derived stem cells(MenSCs)with endometriosis(EMT).Methods:EMT MenSCs(E-MenSCs)and healthy MenSCs(H-MenSCs)were isolated from the menstrual blood of patients with EMT and healthy female participants,respectively.We identified their stem cells’characteristics via adipogenic and osteogenic differentiation.Twelve male SpragueeDawley rats received 0.9% NaCl and HP-dispensing granules by gastric irrigation to prepare blank serum and medicated serum,respectively.We used serum concentrations of 5%,10%,and 20%,each at administered times of 12,24,and 48 h to select the best condition.These cells were divided into three groups:blank serum of the control group,blank serum of the model group,and medicated serum of the HP group.H-MenSCs were used in the control group,while E-MenSCs were used in the model and HP groups.We analyzed cell viability using a cell counting kit-8 assay,observed cell morphology,evaluated the amounts of auto-phagosomes and autolysosomes by transmission electron microscopy,and detected the protein expression of autophagy markers(LC3-II and Beclin1)by Western blot.Results:E-MenSCs and H-MenSCs became long fusiform with a diffuse radial pattern,forming lipid droplets and calcium nodules after adipogenic and osteogenic differentiation.We then used the best conditiond 20% serum and 48 hdfor the subsequent experiments.In contrast to the model group,the HP group exhibited lower cell viability(=0.007),larger amounts of autophagosomes and autolysosomes(P<0.001 and P=0.001,respectively),and higher expression of LC3-II and Beclin1(P=0.021 and P=0.019,respectively).Conclusion:Hupo powder can promote autophagy in E-MenSCs,which might be one of the mechanisms underlying its therapeutic effects.

Langerhans cell histiocytosis masquerading as acute appendicitis: Case report and review

Langerhans cell histiocytosis(LCH) is a rare syndrome characterized by unifocal,multifocal unisystem,or disseminated/multi-system disease that commonly involves the bone,skin,lymph nodes,pituitary,or sometimes lung(almost exclusively in smokers) causing a variety of symptoms from rashes and bone lesions to diabetes insipidus or pulmonary infiltrates.We present a previously unreported case of gastrointestinal LCH as well as a novel characteristic lesion affecting the colon of a young woman who presented with signs and symptoms mimicking acute on chronic appendicitis.Immunohistochemical analysis of appendectomy specimen and nodular specimens on colonoscopy demonstrated S-100,CD1a,and langerin reactivity.The patient underwent systemic chemotherapy with cytarabine and demonstrated excellent response to therapy.

十精丸对光老化大鼠皮肤LCs及炎症因子水平影响的研究

目的:探讨十精丸对于光老化大鼠皮肤朗格汉斯细胞(Langerhanscell,LCs)及体内炎症因子水平的影响。方法:SD大鼠,雌性50只,随机分为模型组、阳性对照组、十精丸低剂量组、十精丸中剂量组、十精丸高剂量组,每组10只。建立皮肤光老化模型,同时给予十精丸干预治疗。实验结束后,通过免疫组化法对大鼠皮肤LCs数量及形态进行观察,病理切片观察皮肤炎性浸润程度,ELISA方法检测皮肤组织和血清中白介素-6(Interleukin-6,IL-6)、肿瘤坏死因子-α(Tumor necrosisfactor-α,TNF-α)含量。结果:免疫组化结果显示,与模型组相比,十精丸各剂量组表皮LCs细胞数目均有所增加,胞体形态改善,树突结构形成增多,细胞间连接紧密。其中十精丸高剂量组改善效果较为显著。病理切片结果显示,十精丸各剂量组大鼠皮肤炎性细胞浸润程度有所改善,十精丸高剂量组改善程度较为明显。ELISA检测结果显示,十精丸各剂量组皮肤组织匀浆及血清中炎症因子IL-6、TNF-α含量均有所下降。其中十精丸中、高剂量组下降明显,差异有统计学意义(P<0.01),与阳性对照组比较,差异无统计学意义(P>0.05)。结论:十精丸能增加光老化大鼠皮肤组织中LCs细胞数量,改善LCs细胞异常形态,减轻大鼠皮肤组织的炎性细胞浸润程度,降低大鼠皮肤组织及血清中炎症因子IL-6、TNF-α的含量,表明十精丸能提高皮肤免疫功能,抑制炎症因子,从而修复皮肤功能,改善皮肤衰老状态。

A NEW LOOK AT LANGERHANS CELL HISTIOCYTOSIS: REVIEW OF A SERIES OF 55 CASES

Objective: Langerhans cell histiocytosis (LCH) has been well described only in children. We analyzed the characteristics, reactivation, and outcome of LCH in a cohort of 55 patients across all ages. Methods: We reviewed the records of all patients with LCH treated at a single institute between Jan. 1974 and May 1998. Results: The 55 patients were 2 to 67 years of age (median, 31 years) at the time of diagnosis, and 85.5% were male. Forty patients (72.7%) had single-system LCH; Fifteen (27.3%) had multisystem disease. The head and neck was the most frequent tumor site (63.6%). LCH was not found in organs at risk of involvement (liver, spleen, bone marrow, and lungs). The frequency of bony invasion (23.6% overall) differed significantly according to age ≤15 years (66.7%) vs. age >15 years (11.6%) (P=0.0005). At a median follow-up of 12 years, no patient died of LCH. The 5, 10-year survival estimates were 100%. The 5, 10-year disease-free survival estimates were 70.9% and 58.4%. The 5-year disease-free survival estimate was 58.3% for age ≤ 15 years vs. 74.4% for age >15 years (P=0.83) and 75% for single-system disease vs. 60% for multisystem disease (P=0.13). LCH was reactivated in 43.6% of patients, with a median of 14 months (range, 2-180 months). Three patients with recurrent disease experienced spontaneous remission. At the time of the most recent follow-up, 23.6% of survivors had active disease. Conclusion: LCH is not found exclusively in children and adolescents. The frequency of bone invasion is inversely related to age. Reactivation is very common regardless of the type of treatment, but the prognosis is generally good.

Atlanto-axial langerhans cell histiocytosis in a child presented as torticollis

Langerhans cell histiocytosis(LCH) is a rare condition mostly seen in children and adolescents. Eosinophilic granuloma(EG) is one of its three clinical entities and is considered as a benign osteolytic lesion. Many reports of patients with spine histiocytosis are well documented in the literature but it is not the case of atlantoaxial localization. We report here a new observation of atlantoaxial LCH in a 4-year-old boy revealed by persistent torticollis. He was successfully treated with systemic chemotherapy and surgery. Inter-body fusion packed by autologous iliac bone was performed with resolution of his symptoms. It is known that conservative treatment is usually sufficient and surgery should be reserved for major neurologic defects in spine EG. In atlantoaxial lesion, surgical treatment should be frequently considered.

Characteristics of multiple nodules in a patient with pulmonary Langerhans cell histiocytosis: A case report

BACKGROUND The common computed tomography findings of pulmonary Langerhans cell histiocytosis (PLCH) are multiple cysts and micronodules predominantly in middle to upper lung lobes.Non-cystic nodules and large nodules are atypical findings of PLCH.CASE SUMMARY The patient was a 48-year-old Japanese man with a smoking history (20 cigarettes/d,28 years) and no symptoms.Multiple nodules existed in all lung lobes,predominantly in the right lower lobe.Some nodules seemed to be distributed randomly,and others were adjacent to bronchus.Most nodules were solid;some small ones were cystic.The largest nodule was 22 mm in diameter.Although metastatic lung tumors were suspected,thoracoscopic lung biopsy led to the diagnosis of PLCH.At 6 months after he quit smoking,all nodules had almost disappeared.We investigated the characteristics of nodules at diagnosis in detail.Of 349 nodules in total,116 were in upper and 199 were in lower lobes.Ninety-six (27.5%) were cystic;the remaining 253 (72.5%) were non-cystic.The prevalence of cystic nodules was higher in upper lobes than in lower lobes (right upper 37.5% vs lower 18.2%,P = 0.0068;left upper 48.1% vs lower 24.4%,P = 0.0078).The average size (dia.) of cystic nodules was smaller than that of noncystic nodules (5.03 mm vs 7.40 mm,respectively,P < 0.0001).CONCLUSION Although multiple non-cystic nodules including large nodules (over 20 mm) are atypical,PLCH should be included in differential diagnoses.The presence of small cystic nodules predominantly in upper lobes and asymptomatic situation are also important for differential diagnoses to distinguish from metastatic cancers.

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.

Hepatic Langerhans cell histiocytosis:A review

Hepatic Langerhans cell histiocytosis(LCH)is characterized by proliferation and accumulation of Langerhans cells in the liver,causing liver dysfunction or forming a mass lesion.The liver can be involved in isolation,or be affected along with other organs.A common clinical hepatic presentation is cholestasis with pruritis,fatigue and direct hyperbilirubinemia.In late stages,there may be hypoalbuminemia.Liver biopsy may be required for the diagnosis of hepatic LCH.Histologic finding may be diverse,including lobular Langerhans cell infiltrate with mixed inflammatory background,primary biliary cholangitis-like pattern,sclerosing cholangitis-like pattern,and even cirrhosis at later stages.Because of its non-specific injury patterns with broad differential diagnosis,establishing a diagnosis of hepatic LCH can be challenging.Hepatic LCH can easily be missed unless this diagnosis is considered at the time of biopsy interpretation.A definitive diagnosis relies on positive staining with CD1a and S100 antigen.Liver involvement is a high risk feature in LCH.The overall prognosis of hepatic LCH is poor.Treating at an early stage may improve the outcome.Systemic chemotherapy is the mainstay of treatment and liver transplantation may be offered.New molecular markers involved in pathogenesis of LCH are being explored with a potential for targeted therapy.However,further studies are needed to improve outcome.

Langerhans cell histiocytosis involving only the thymus in an adult: A case report

BACKGROUND Langerhans cell histiocytosis(LCH)is a rare disease of unknown etiology.LCH involving the thymus is mainly seen in pediatric patients and is extremely rare in adults.In this report,we describe a rare case of LCH originating from the thymus in an adult.CASE SUMMARY A 56-year-old man was admitted in April 2022 with complaints of intermittent dizziness since 2020,which had worsened in the previous 10 d.The physical chest examination was negative,and there was a history of hypertension for>2 years.Chest computed tomography showed a nodular soft tissue density shadow in the anterior mediastinum measuring approximately 13 mm×9 mm×8 mm.Postoperative pathological findings confirmed the diagnosis of LCH.CONCLUSION It is challenging to differentiate LCH involving the thymus from thymoma in imaging features.Pathological biopsy remains the gold standard when an anterior mediastinal occupying lesion is found.

Multisystem involvement Langerhans cell histiocytosis in an adult:A case report

BACKGROUND Langerhans cell histiocytosis(LCH)is a rare condition wherein Langerhans cells proliferate abnormally,adversely impacting organs including lymph nodes,bones,skin,lungs,and pituitary gland.The LCH disease course varies widely among patients from a self-limiting condition to one that progresses rapidly and culminates in death.It is uncommon for multisystem LCH to be observed in adults.Herein we describe a woman suffering from multi-system LCH involvement.CASE SUMMARY A 37-year old Chinese woman was admitted to the hospital in June 2019 suffering from dyspnea that had progressed over the course of 5 years.Her medical history included:central diabetes insipidus(DI)that had been treated via radiotherapy,desmopressin acetate,and bromocriptine;bilateral pneumothorax with two surgeries having been performed to remove bullae;and autoimmune hepatitis that had been unsuccessfully treated using a combination of methylprednisolone and mycophenolate mofetil.A chest computed tomography(CT)scan revealed the presence of multiple pulmonary cysts of varying sizes.We re-analyzed right pulmonary bullae samples that had been removed in 2014,performed a systematic 18 F-FDG PET/CT analysis,and convened a multidisciplinary medical team to diagnose and treat this patient.As a result,we were able to eventually diagnose this patient with LCH that was not associated with BRAF-V600 E mutations.CONCLUSION We hope to emphasize the importance of systemic evaluation and of cooperation between multidisciplinary physicians with the goal of improving awareness and detection of this orphan disease.

Pulmonary Langerhans cell histiocytosis in adults: A case report

BACKGROUND Langerhans cell histiocytosis (LCH) is a rare disease of unknown aetiology. While it may affect any organ of the body, few cases of solitary lung involvement are published in the literature. Here, we report a rare case of pulmonary LCH (PLCH) in an adult. CASE SUMMARY A 52-year-old male presented to hospital in July 2018 with complaints of progressively worsening cough with sputum, breathlessness, easy fatigability, and loss of appetite since 2016, and a 32-year history of heavy cigarette smoking (average 30 cigarettes/d). Physical examination showed only weakened breathing sounds and wheezing during lung auscultation. Chest computed tomography (CT) showed irregular micronodules and multiple thin-walled small holes. Respiratory function tests showed a slight decrease. Ultrasonic cardiogram showed mild tricuspid regurgitation and no pulmonary hypertension. Fibreoptic bronchoscopy was performed with transbronchial biopsies from the basal segment of right lower lobe. LCH was confirmed by immunohistochemistry. The final diagnosis was PLCH without extra-pulmonary involvement. We suggested smoking cessation treatment. A 3-mo follow-up chest CT scan showed clear absorption of the nodule and thin-walled small holes. The symptoms of cough and phlegm had improved markedly and appetite had improved. There was no obvious dyspnoea. CONCLUSION Imaging manifestations of nodules, cavitating nodules, and thick-walled or thinwalled cysts prompted suspicion of PLCH and lung biopsy for diagnosis.

Prevention of core cell damage in isolated islets of Langerhans by low temperature preconditioning

AIM: To study the core cell damage in isolated islets of Langerhans and its prevention by low temperature preconditioning (26 ℃).METHODS: Islets were cultured at 37 ℃ for 7-14 d after isolation, and then at 26 ℃ for 2, 4 and 7 d before additional culture at 37 ℃ for another 7 d. Core cell damage in the isolated islets was monitored by video-microscopy and analyzed quantitatively by use of a computer-assisted image analysis system. The analysis included daily measurement of the diameter and the area of the isolated islets and the area of the core cell damage that developed in those islets over time during culture. Histology and TdT-mediated dUTP-biotin nick end labeling (TUNEL) assay were used to characterize the cell damage and to monitor islet function.RESULTS: Microscopic analysis showed that during the 7 to 14 d of culture at 37 ℃, core cell damage occurred in the larger islets with diameters ＞200 μm, which included both necrotic and apoptotic cell death. Low temperature (26 ℃) culture could prevent core cell damage of isolated islets. The 7-d culture procedure at 26 ℃ could inhibit most of the core cell (excluding diameters＞300 μm) damages when the islets were re-warmed at 37 ℃.CONCLUSION: Our results indicate that core cell damage within isolated islets of Langerhans correlates with the size of islets. Low temperature (26 ℃) culture can prevent core cell damage in isolated islets, and successfully precondition these islets for incubation at 37 ℃. These novel findings may help to understand the pathophysiology of early loss of islet tissue after transplantation, and may provide a new strategy to improve graft function in the clinical setting of islet transplantation.

A rare case of langerhans cell histiocytosis of the gastrointestinal tract

Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the proliferation of specialized,bone marrow-derived langerhans cells and mature eosinophils.The clinical spectrum ranges from an acute,fulminant,disseminated disease called LettererSiwe disease to solitary or few,indolent and chronic lesions of the bone or other organs called eosinophilic granuloma.Involvement of the gastrointestinal tract is very rare in LCH.We present the case of a 53-yearold woman referred by her primary care physician for a screening colonoscopy.A single sessile polyp,measuring 4 mm in size,was found in the rectum.Histopathological examination revealed that the lesion was relatively well circumscribed and comprised mainly a mixture of polygonal cells with moderate-to-abundant pink slightly granular cytoplasm.The nuclei within these cells had frequent grooves and were occasionally folded.Immunohistochemical staining was positive for CD1a which confirmed the diagnosis of LCH.On further workup,there was no evidence of involvement of any other organ.On follow up colonoscopy one year later,there was no evidence of disease recurrence.Review of the published literature revealed that LCH presenting as solitary colonic polyp is rare.However,with the increas-ing rates of screening colonoscopy,more colonic polyps may be identified as LCH on histopathology.This underscores the importance of recognizing this rare condition and ensuring proper follow-up to rule out systemic disease.

Ocular Trauma as the First Presentation of Langerhans Cell Histiocytosis

Purpose:Ocular trauma occurs disproportionately in children on an annual basis.However,because of this frequency,other diagnoses,.such as orbital neoplasms,.can easily be ignored.Methods:We report on a two-year-old boy who presented with a dark purple,irregularly shaped lesion on his lower left eyelid.The patient had suffered injuries twice to that area.Results:Axial computed tomography images demonstrated an ill-defined and inhomogeneous soft tissue mass in the lateral and posterior region of the left orbit,.with bony destruction and absorption of the adjacent orbital wall..Magnetic resonance imaging of the orbit showed a lesion involving the greater sphenoidal wing,the supraorbital wall,and the frontal area.Enlarged nuchal and inguinal lymph nodes were detected by sonographic examination.Histopathological examination of a surgical biopsy showed an accumulation of characteristic Langerhans cells.The diagnosis of Langerhans cell histiocytosis was confirmed by immunohistochemical examinations positive for CD1a and SP-100.Conclusion:Orbital Langerhans cell histiocytosis is rarely encountered in ophthalmic practice,.so the ophthalmologist needs to be familiar with its presentation and work-up,.and has to be aware of possible OLCH diagnosis when a child(or even an adult)presents with prolonged and persistent eyelid edema,.even with a history of ocular trauma.

Current perspectives on the role of liver transplantation for Langerhans cell histiocytosis:A narrative review

Langerhans cell histiocytosis(LCH)is a malignant disease of the histiocytes involving various organ systems.The spectrum of liver involvement in LCH ranges from mild transaminitis to end-stage liver disease.The hallmark of hepatic LCH is secondary sclerosing cholangitis,which manifests due to a progressive destruction of the biliary tree by malignant histiocytes.Chemotherapy remains the mainstay of treatment for active LCH.Early recognition,diagnosis and a systematic approach to the management of LCH can ameliorate the disease process.Nonetheless,the liver involvement in these patients may progress despite the LCH being in remission.Liver transplantation(LT)remains central in the management of such patients.Various facets of the management of LCH,especially those with liver involvement remain unclear.Furthermore,aspects of LT in LCH with regards to the indication,timing and post-LT management,including immunosuppression and adjuvant therapy,remain undefined.This review summarises the current evidence and discusses the practical aspects of the role of LT in the management of LCH.