Langerhans-cell

  • 文章类型: Case Reports
    嗜酸性肉芽肿(EG),朗格汉斯细胞组织细胞增生症(LCH)的亚型,单骨形式,是一种罕见的疾病,其特征是孤立的骨病变。这种情况伴有硬膜外血肿(EDH)更为罕见。这种情况是独特的,因为它是第一个涉及癫痫发作后延迟EDH的病例。我们描述了EG伴随EDH的显着例子,并考虑了这种共病的罕见性。一名32个月大的男孩在头部轻伤后发展出快速增长的头骨。在活检的手术准备过程中,病人经历了一次抽搐。癫痫发作后的成像显示肿块附近有EDH。切除肿块,确认为EG,但利润率为正。患者在全身骨骼评估后接受化疗,根据组织细胞增生症协会建立的LCHIII方案。EG是一种罕见的肿瘤,通常表现为颅骨上的无痛生长,随着时间的推移逐渐扩大。EG和EDH之间的相关性非常罕见,只有几个记录在案的案例。此案例研究强调了在颅骨肿块扩大的鉴别诊断中考虑EG的重要性,即使与EDH相关联。及时的诊断和治疗可以预防严重的并发症并改善患者的预后。
    Eosinophilic granuloma (EG), a subtype of Langerhans cell histiocytosis (LCH), the monostotic form, is a rare condition characterized by a solitary bone lesion. It is even more unusual for this condition to be accompanied by an epidural hematoma (EDH). This case is unique in that it is the first to involve delayed EDH following a seizure. We describe a remarkable example of EG accompanied by an EDH and consider the rarity of this comorbidity. A 32-month-old boy developed a rapidly growing skull mass following a minor head injury. During surgical preparation for a biopsy, the patient experienced a single convulsion. Imaging following the seizure revealed an EDH in the vicinity of the mass. The mass was excised and confirmed to be an EG, but with positive margins. The patient underwent chemotherapy after systemic skeletal evaluation, in accordance with the LCH III protocol established by the Histiocytosis Society. EG is a rare neoplasm that typically presents as a painless growth on the skull that gradually enlarges over time. The correlation between EG and EDH is exceedingly uncommon, with only a few documented cases. This case study underscores the significance of considering EG in the differential diagnosis of an expanding cranium mass, even when associated with EDH. Prompt diagnosis and treatment can prevent serious complications and improve patient outcomes.
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  • 文章类型: Case Reports
    朗格汉斯细胞组织细胞增生症(LCH)是成人的罕见疾病,特别是当它仅限于头骨的一个区域时,称为单发颅骨受累。在这个案例报告中,我们提出了一个独特的LCH影响顶骨的例子,伴有脓液引流瘘。这是一个罕见的和不寻常的演示在这个地方,这在医学文献中几乎没有报道。一名30岁的女性,以前没有合并症,她的头痛症状持续了一年。她的头皮也有肿胀,有3周的黄色分泌物,但没有观察到神经系统问题。放射学显示颅骨变薄,沿着内表的边缘参差不齐,多个局灶性侵蚀,以及累及上覆的软组织和骨死骨组织。患者接受了双顶开颅手术并切除了病变。组织病理学报告显示为LCH。经过8个月的随访,没有复发。可以通过完全切除病变来实现LCH对孤立性颅骨受累的处理,并伪装成头皮感染。导致有利的结果。
    Langerhans cell histiocytosis (LCH) is a rare condition in adults, especially when it is limited to a single area of the skull, known as solitary calvarial involvement. In this case report, we present a unique instance of LCH affecting the parietal bone with a pus-draining fistula. This is a rare and unusual presentation at this location, which has been scarcely reported in medical literature. A 30-year-old woman with no prior comorbidity presented with complaints of headache that persisted for a year. She also had swelling on her scalp and a yellowish discharge for 3 weeks, but no neurological problems were observed. Radiology revealed thinning of the calvaria, with ragged margins along the inner table, multiple focal erosions, and involvement of overlying soft tissue and bony sequestrum. The patient underwent biparietal craniotomy and excision of the lesion. The histopathology report showed LCH. After 8 months of follow-up, there was no recurrence. The management of solitary calvarial involvement by LCH with masquerading presentation as a scalp infection can be achieved through complete excision of the lesions, resulting in a favorable outcome.
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  • 文章类型: English Abstract
    Histiocytosis comprises a heterogeneous group of inflammatory diseases whose main cellular components are dendritic cells and macrophages. The inflammatory infiltrate can affect the skin and other organs and the clinical outcome varies from mild to fatal depending on the involved cell subset and multisystemic compromise. Delay in diagnosis may occur due to its non-specific presentation and to a low suspicion on the part of the clinician. We report the case of an infant who despite multiple consultations with nonspecific but characteristic symptoms of the disease was only finally diagnosed thanks to histopathological findings.
    La histiocitosis de células de Langerhans comprende un grupo heterogéneo de enfermedades inflamatorias cuyos principales componentes celulares son las células dendríticas y los macrófagos. El infiltrado inflamatorio puede afectar la piel y otros órganos, y el resultado clínico varía de leve a letal, dependiendo del subconjunto de células involucradas y el compromiso multisistémico. La demora en el diagnóstico puede ocurrir debido a su presentación inespecífica y a que los médicos tratantes no suelen sospecharla. Se reporta el caso de una lactante mayor a la cual, a pesar de múltiples consultas con síntomas inespecíficos pero característicos de la enfermedad, solamente se le pudo hacer el diagnóstico gracias a los hallazgos histopatológicos.
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  • 文章类型: Journal Article
    Smoking-associated interstitial lung disease manifests as several heterogeneous disorders involving the airways, pleura, and lung parenchyma with various radiological patterns. The clinical history, radiologic, and pathologic findings are important to distinguish these more uncommon diseases. A multidisciplinary approach is recommended for diagnosis and to manage these conditions appropriately.
    This review provides an overview of the epidemiology, risk factors, pathogenesis, clinical features, diagnosis, and treatment of acute eosinophilic pneumonia, e-cigarettes, or vaping associated lung injury, respiratory bronchiolitis interstitial lung disease, desquamative interstitial pneumonitis, pulmonary Langerhans cell histiocytosis, idiopathic pulmonary fibrosis, and combined pulmonary fibrosis emphysema.
    Cigarette smoking is associated with a variety of pathologic conditions that affect the airways and lungs. E-cigarette use and vaping present new challenges to the clinician. Consensus between the clinical, radiographic, and pathologic findings is important in identifying and differentiating between the various entities to properly diagnose smoking-related interstitial lung diseases discussed in this review.
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  • 文章类型: Case Reports
    Bone necrosis of the jaw is a serious condition with a broad differential diagnosis of pathologies such as cutaneous histiocytosis, bone metastases or malignant tumours. In addition to the most common cause, medication related osteonecrosis of the jaw (MRONJ), one must consider a number of other causes, such as histiocytosis. Langerhans cell histiocytosis (LCH) is a histiocytic disorder with a large spectrum of clinical manifestations and with possible involvement of a variety of organs. This case shows the importance of an early detection of this rare disease in order to prevent further spreading. Even if an initial diagnosis in the oral cavity is rare, dentists should be aware of this disease.
    The presented case describes a patient who was referred for evaluation and treatment due to exposed bone and extensive osteolysis in the region of the upper and lower jaw. After biopsy and diagnosis of LCH, the patient was treated with systemic therapy, achieved remission and is disease free after a 2 year of follow up.
    This case report illustrates that when dealing with unclear osteolytic changes of the jawbone, Langerhans cell histiocytosis must be taken into consideration in the differential diagnosis and biopsy must be performed in case of suspicion.
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    文章类型: Journal Article
    UNASSIGNED: Langerhans cell histiocytosis (LCH) is a rare histiocytic proliferative disorder of unknown etiology and mainly affects young children. The histological feature is granuloma-like proliferation of langerhans-type dendritic cells. Although the possible role of viruses such as Epstein-Barr virus (EBV, Human Herpes virus -4), Human Herpes virus-6 (HHV-6), Herpes Simplex virus (HSV) types 1 and 2 and Cytomegalovirus (CMV, Human Herpes virus-5) is suggested in the pathogenesis of LCH by some investigators, its exact pathophysiology has not been cleared yet. In this study, we investigated the presence of HSV types 1 and 2 in Iranian children with LCH.
    UNASSIGNED: In this retrospective study, we investigated the prevalence of presence of HSV types 1 and 2 (in 30 patients with LCH), using paraffin-embedded tissue samples and 30 age and tissue-matched controls (operated for reasons other than infectious diseases) from the Department of Pediatric Pathology, Tehran, Iran, by nested Polymerase Chain reaction method. No ethical issues arose in the study, because only the pathology reports were reviewed and patients were anonymous.
    UNASSIGNED: We failed to find HSV types 1 and 2 DNA in any of the 30 patients with LCH or the control group.
    UNASSIGNED: According to our findings, HSV types 1 and 2 do not appear to have any etiologic role in the pathogenesis of LCH in Iranian children. These results are in accordance with previous investigations with negative findings.
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  • 文章类型: Case Reports
    先天性自愈性网状组织细胞增生症(CSHRH)是一种罕见的,皮肤,自我限制形式的朗格汉斯细胞组织细胞增生症。而多发性病变是常见的,孤立性病变是罕见的。一个14天大的新生儿,5-mm,椭圆形,带红色,从出生以来就存在的左侧大腿上有皮的丘疹被侵蚀。未发现系统参与。组织病理学显示大组织细胞密集浸润,真皮中嗜酸性粒细胞和淋巴细胞散在。免疫组织化学显示S-100和CD1a阳性。两个月后,皮肤病变自发消退,没有复发和皮肤外受累的证据。根据特征性的临床过程和典型的组织病理学发现,诊断为单发CSHRH.
    Congenital self-healing reticulohistiocytosis (CSHRH) is a rare, cutaneous, self-limited form of Langerhans cell histiocytosis. Whereas multiple lesions are common, a solitary lesion is rare. A 14-day-old neonate presented with a solitary, 5-mm, oval, reddish, and eroded papule with crust on the left thigh that had existed since birth. No systemic involvement was found. Histopathology revealed dense infiltration of large histiocytes with scattered eosinophils and lymphocytes in the dermis. Immunohistochemistry showed S-100 and CD1a positivity. Two months later, the skin lesion involuted spontaneously, without evidence of recurrence and extracutaneous involvement. On the basis of the characteristic clinical course and typical histopathological findings, a diagnosis of solitary CSHRH was made.
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