Langerhans’ Cell Histiocytosis

朗格汉斯细胞组织细胞增生症
  • 文章类型: Journal Article
    多焦朗格汉斯\'细胞组织细胞增生症是一种罕见的疾病,可影响多个器官并在各种情况下表现出来。虽然这种情况更常见于儿童,它也可以发生在成人。
    一名43岁女性出现顽固性耳漏,左中颈区域颈部有橡胶状肿块,以及左侧顶叶区域瘙痒的湿疹样病变。最终切除了耳部病变,组织病理学检查证实了朗格汉斯组织细胞增生症的诊断。
    虽然在成人中很少见,朗格汉斯组织细胞增生症应被视为耳道息肉的鉴别诊断之一。如果被诊断,应该继续治疗。
    UNASSIGNED: Multifocal Langerhans\' cell histocytosis is a rare condition that can affect multiple organs and manifest in various scenarios. While the condition is more commonly found in children, it can also occur in adults.
    UNASSIGNED: A 43-year-old female presented with refractory otorrhea and had a rubbery neck mass in the left mid-cervical area, as well as an itchy eczematoid lesion in the left parietal area. The otic lesion was eventually resected, and histopathologic examination confirmed the diagnosis of Langerhans histiocytosis.
    UNASSIGNED: Although rare in adults, Langerhans histiocytosis should be considered as one of the differential diagnoses for ear canal polyps. If diagnosed, medical treatment should be pursued.
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  • 文章类型: Journal Article
    我们介绍了一个孤立的朗格汉斯细胞组织细胞增生症的6岁儿童,他表现为右耳溢,耳后肿胀和听力损失。术前临时诊断为胆脂瘤,每次手术鉴别诊断为横纹肌肉瘤,最终组织学和免疫组织化学诊断为朗格汉斯细胞组织细胞增生症。
    We present a case of isolated Langerhans\' cell histiocytosis in a six year old child who presented with right otorrhoea, retro-aural swelling and hearing loss. The preoperative provisional diagnosis was cholesteatoma, per operative differential diagnosis was rhabdomyosarcoma and final histological and immunohistochemistry diagnosis was Langerhans\' cell histiocytosis.
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  • 文章类型: Journal Article
    Langerhans\' cell histiocytosis (LCH) is a rare disease observed in childhood characterized by the proliferation of Langerhans\' cells resulting in focal or systemic manifestations (including the bones). Here, we present a pediatric case with a localized biopsy-proven LCH, who underwent progression from solitary to multifocal form detected on bone scintigraphy and single photon emission computerized tomography/computed tomography (SPECT/CT) performed within four months. Emphasizing on localized bone pain (predictive of osseous LCH) and local tenderness and swelling usually guides the nuclear physician to perform additional SPECT/CT with presumably an improvement of the diagnostic accuracy as demonstrated in our case.
    Langerhans hücreli histiyositoz (LHH), genellikle çocukluk çağında görülen, Langerhans hücrelerinin çoğalmasıyla birlikte kemikler de dahil olmak üzere lokal veya sistemik tutulum ile seyreden nadir bir hastalıktır. Burada, soliter olup dört ay sonra yapılan kemik sintigrafisi ve tek foton emisyon tomografisi/bilgisayarlı tomografide (SPECT/BT) multifokal forma ilerlediği saptanan, biyopsi ile kanıtlanmış LHH tanılı pediatrik bir olgu sunulmaktadır. Bu olguda görülmektedir ki; lokalize kemik ağrısı şikayeti olan ve/veya fizik muayenede lokal hassasiyet ve şişme gibi bulguları olan hastalarda, ilgili alanın ek SPECT/BT görüntülerinin alınması ile tanısal doğrulukta iyileşme sağlamaktadır.
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  • 文章类型: Journal Article
    Langerhans cell histiocytosis (LCH) is a common cause of multifocal lytic skeletal lesions in children. However, multifocal osseous tuberculosis can affect children and mimics LCH on imaging, especially in endemic regions. We report cases with atypical manifestations of multifocal osseous tuberculosis which were presumptively diagnosed as LCH. The findings of our series of cases suggest that on computed tomography (CT) irregular sclerotic margins, abscess formation, sclerosis of involved bone, and button sequestrum point toward a diagnosis of multifocal osseous tuberculosis, especially in endemic regions.
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  • 文章类型: Journal Article
    We present a case of papillary thyroid cancer (post-thyroidectomy status) on regular treatment with suppressive Levothyroxine therapy. On follow-up at 6 months after radioactive iodine ablation for remnant thyroid tissue, her thyroglobulin, and anti-thyroglobulin levels were 0.06 ng/ml and 670 IU/ml, respectively. Low-dose whole-body I-131 scan was negative. To look for the cause of isolated increased anti-thyroglobulin level, a whole-body 18F-FDG PET/CT was done which revealed multiple FDG-avid lytic skeletal lesions suggestive of metastases. For confirmation of diagnosis, 18F-FDG PET/CT-guided metabolic biopsy was done, which revealed Langerhans\' cell histiocytosis on histopathological examination.
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  • 文章类型: Journal Article
    Langerhans\' cell histiocytosis (LCH) is a rare disease of unknown etiology and variable biologic behavior and course. Almost every organ can be affected; however, it frequently involves the bones, lungs, skin and lymph nodes. LCH exhibits a particular predilection for involvement of the hypothalamo-pituitary axis resulting in diabetes insipidus and/or other anterior pituitary deficiencies. Although LCH can sometimes resolve spontaneously, it usually follows a chronic course and systemic treatment is mandatory for the management of multisystem disease. However, a significant number of patients will develop at least one permanent sequela as a consequence of the disease, with diabetes insipidus being the most common. A multidisciplinary approach is always needed in diagnosis, treatment and follow-up of all patients with LCH.
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  • 文章类型: Case Reports
    BACKGROUND: Langerhans\' cell histiocytosis (LCH) is a proliferative disorder of Langerhans cells, which is seen extremely rarely in adults. Conventional imaging modalities, such as skeletal surveys and bone scans, were accepted to be standard methods for diagnosis; however, 18-fluorodeoxyglucose positron emission tomography (PET-CT) has been increasingly used.
    METHODS: We report on a 33-year-old female patient with disseminated LCH treated with radiotherapy and systemic chemotherapy where PET-CT has been used for defining the extent of the disease, RT planning and assessment of treatment response during follow-up.
    RESULTS: The patient was treated with 24 Gy 3-dimensional conformal radiotherapy (RT), given as 2 Gy a day, 5 days a week. The patient was also treated with systemic prednisolone 20 mg/m(2), concurrently. The chemotherapeutic regimen switched to cytosine-arabinoside with a dose of 100 mg/m(2) subcutaneously daily for 4 days, vincristine 1.5 mg/m(2) given on the 1st day and prednisolone 20 mg/m(2) for 4 cycles. After local RT with adjuvant chemotherapy, the patient was alive for 54 months and remained disease-free at last visit.
    CONCLUSIONS: RT is a treatment choice in multi-system LCH as well as solitary lesions. Low-dose RT is adequate to control large masses of LCH including soft tissue and lymph nodes.
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  • 文章类型: Evaluation Study
    OBJECTIVE: Pulmonary Langerhans cell histiocytosis (PLCH) is a rare respiratory disease closely associated with smoking. The aim of the study is to know the reality of PLCH in the Catalonian population.
    METHODS: We included all patients diagnosed of PLCH in 8 different Catalonian hospitals from April 2011 to December 2012. Epidemiologic, clinical, radiological, functional, diagnostic and therapeutic variables were collected, both at the beginning and at the last visit of the process.
    RESULTS: Thirty two patients were included. Overall, patients were middle-aged (38 years), with no gender predominance. Most of them were smokers (71.9%). The main pulmonary function values (forced vital capacity 75.9%, forced expiratory volume in one second 68.9%, diffusing capacity of the lung for carbon monoxide 59.6%) and radiological findings (cysts 89.7%, nodules 60.3%) showed no significant changes during evolution. However, the disease progressed with a worsening lung function in 34.4% of cases. The analysis of the functional outcome (forced vital capacity, forced expiratory volume in one second, and diffusing capacity of the lung for carbon monoxide) did not reveal significant differences according to the persistence or not of smoking (P<0.05).
    CONCLUSIONS: Our patients with PLCH had similar clinical and radiological characteristics to those described in other populations. The results observed regarding the relationship with smoking suggest that there may be other factors beyond smoking, which influence on the evolution of the disease. It therefore seems advisable to design prospective studies to investigate this aspect.
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    文章类型: Case Reports
    Eosinophilic granuloma is a rare disease which is difficult to diagnose clinically and radiographically. Localized Langerhans\' cell histiocytosis, previously known as eosinophilic granuloma, mainly affects the skull, mandible, vertebrae, pelvis and ribs in children and the long bones of adults. We present a case report of a female who developed pain and swelling over the left mandibular region, and was later diagnosed as eosinophilic granuloma, which after administration of intralesional corticosteroid with surgical enucleation showed positive response. This disease is of importance to dental professionals because early clinical signs can occur in the jaw and can cause extensive destruction of the periodontal tissues and bone. The purpose of this case report is to describe a case of eosinophilic granuloma with emphasis on conservative approach for the treatment and the radiographic changes observed during and after the treatment.
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