Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 28-year-old woman was admitted to our Hospital with a chief complaint of progressive gingival swelling and loosening of teeth over about a year. According to past history, she had received total thyroidectomy 2 years previously due to thyromegaly. The thyroidectomy specimen was at first interpreted as 'poorly differentiated carcinoma of the thyroid'. One year ago, she began to be aware of gingival swelling and loosening of teeth. A gum biopsy was taken and the pathologic features were similar to her 'thyroid carcinoma'. Subsequent investigations, including immunohistochemical stain, showed the gum was heavily infiltrated with histiocyte-like Langerhans' cells which were positive for S-100 protein. Ultrastructural examination of the cells under electron microscope revealed many typical intra-cytoplasmic Birbeck granules. Langerhans' cell histiocytosis was diagnosed. Langerhans' cell histiocytosis with thyroid involvement is extremely rare and may run a relatively indolent course. Even on a retrospective examination, it may easily be confused with poorly differentiated carcinoma of the thyroid. We suspect that this error may have been made on other occasions and that the occurrence of this condition may be underreported.
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PMID:Langerhans' cell histiocytosis with thyroid involvement masquerading as thyroid carcinoma. 925 74

The entity known as sinus histiocytosis with massive lymphadenopathy (SHML), or Rosai-Dorfman disease (RD disease), is an uncommon benign proliferation of hematopoietic and fibrous tissue that often presents in the head and neck region. Its initial manifestations most often include a roughly symmetric, painless, bilateral cervical adenopathy, although extranodal disease may develop in a minority of patients. The key histologic feature of SHML is the presence of various numbers of large, pale histiocytic cells that contain within their cellular borders apparently engulfed lymphocytes ("emperipolesis"); these distinctive large, pale cells - RD cells - are S-100 protein-positive by immunostaining and so differ from ordinary histiocytes. Despite its sometimes impressive clinical presentation, SHML is a benign and self-limited disease, whose treatment is aimed largely at controlling its local manifestations (most often by surgical therapy). The microscopic differential diagnosis, particularly in extranodal disease, is at times challenging and can include Langerhans' cell histiocytosis, Hodgkin's disease, non-Hodgkin's lymphoma, metastatic carcinoma, and metastatic malignant melanoma.
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PMID:Review of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) of head and neck. 1057 39

We report the case history of a 28-year-old woman who developed synchronous bilateral carcinoma of the breasts, having been diagnosed with multisystem Langerhans' cell histiocytosis in infancy. She had been treated with vinblastine and corticosteroids for 3 years and made a full recovery without late sequelae. We review the association of Langerhans' cell histiocytosis and its treatment with subsequent malignancy, with particular reference to carcinoma of the breast, and discuss the possible causes.
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PMID:Synchronous bilateral carcinoma of the breasts occurring in a young woman with a history of Langerhans' cell histiocytosis in infancy. 1066 33

The ground-glass pattern is a common but nonspecific finding on CT. In certain clinical circumstances, it can suggest a specific diagnosis, indicate a potentially treatable disease, and guide a clinician to an appropriate area for biopsy. A pattern of centrilobular ground-glass nodules is fairly specific for the diagnosis of hypersensitivity pneumonitis with the appropriate clinical history. The tree-in-bud pattern indicates disease affecting the small airways. The differential diagnosis is lengthy; however, the most common process leading to this CT appearance is infection. Although commonly associated with M. tuberculosis, many infectious organisms can produce this pattern. When honeycombing is seen on HRCT, a confident diagnosis of lung fibrosis can be made. The most common causes of interlobular septal thickening on HRCT are pulmonary edema, pulmonary hemorrhage, and lymphangitic spread of cancer, and smooth thickening is characteristic of all three. Diffuse lung cysts in patients who are not immunocompromised generally signify Langerhans' cell histiocytosis, lymphangioleiomyomatosis, or centrilobular emphysema. Centrilobular emphysema can be diagnosed when the centrilobular artery is seen as a small nodular opacity in the center of the cyst. Langerhans' cell histiocytosis is often associated with parenchymal nodules, helping to distinguish it from lymphangioleiomyomatosis. When a nodular pattern is seen on HRCT, the differential diagnosis is very long, but can be narrowed by noting whether the nodules are random, centrilobular, or perilymphatic in distribution. A mosaic pattern of lung attenuation can represent an infiltrative, small airway, or vascular process. The distinction can often be made by noting the size of the pulmonary vessels in the abnormal areas of lung, and whether air trapping is present on expiratory scanning. Computed tomographic signs can be useful indicators of a specific disease process. For instance, the air bronchogram sign indicates that an opacity is intrapulmonary in location, and signals the possibility of two types of neoplasm: lymphoma and bronchioloalveolar cell carcinoma. An air crescent sign indicates recovery of the immune system in an immunocompromised patient with invasive pulmonary aspergillosis. The fallen lung sign is diagnostic of a bronchial transection in the correct clinical setting. The gloved finger sign is very suggestive of allergic bronchopulmonary aspergillosis. The halo sign is highly suggestive of early angioinvasive pulmonary aspergillosis in patients with acute leukemia. When a split pleura sign is seen, the diagnosis is often empyema, although other causes of pleuritis can lead to a similar CT appearance.
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PMID:CT signs and patterns of lung disease. 1169 64

We evaluated the immunohistochemical profile and specificity of CD138 reactivity in 238 specimens from hematopoietic and nonhematopoietic neoplasms. In 91 bone marrow biopsies, CD138 reactivity was observed for nonneoplastic plasma cells, neoplastic plasma cells in multiple myeloma cases (43/43), and the plasmacytic component in lymphoplasmacytic lymphoma cases (4/4). Stromal reactivity was noted in 7 multiple myeloma cases. Of 9 bone marrow specimens involved by metastatic carcinoma, tumor cells were CD138+ in 5 cases; stromal reactivity was noted in 7 cases. Studies of 76 nodal and extranodal lymphomas (B-cell, 49; T-cell, 8; Hodgkin lymphoma, 19), 1 Langerhans cell histiocytosis, and 14 nonneoplastic lymph nodes revealed CD138 reactivity only for nonneoplastic plasma cells, the neoplastic cells of 2 large B-cell lymphomas (immunoblastic type, plasmacytoid features), and the clonal plasmacytic component of 3 of 3 extranodal and 1 nodal marginal zone lymphoma. Evaluation of 56 epithelial and nonepithelial tumors revealed CD138 positivity for neoplastic cells of carcinomas of various types (30/33), frequently with associated stromal reactivity, and for neoplasms of mesenchymal, melanocytic, and other tumor types (12/23). Within the hematopoietic system, CD138 is an excellent marker of plasmacytic differentiation. Based on its broad staining profile, CD138 reactivity for neoplastic cells is not a definitive marker for plasmacytic derivation, unless a hematolymphoid origin has been established.
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PMID:CD138 (syndecan-1), a plasma cell marker immunohistochemical profile in hematopoietic and nonhematopoietic neoplasms. 1498 40

A 57-year-old man who was a heavy smoker was admitted to our hospital for further evaluation of abnormal shadows on a chest X-ray film. Chest radiography and a computed tomography (CT) scan revealed nodular lesions and multiple thin-walled cysts in both lungs. Histopathological examination of one of these cystic lesions showed that the predominant cellular population was Langerhans cells, with the cytoplasm testing positive for S-100 protein and the cell membrane showing a positive reaction for CD1a. The pathological diagnosis was pulmonary Langerhans cell histiocytosis (LCH). A lingual carcinoma that had been detected simultaneously was treated with neoadjuvant therapy and the patient was advised to stop smoking. However, only limited improvement was seen on follow-up chest CT. In view of this, a radical resection of the lingual carcinoma was performed. There was a subsequent dramatic improvement in the pulmonary LCH. Langerhans cells may play a role in the immune response to tumors. In this patient, we suggest the possibility that both the habitual smoking and the lingual carcinoma may have contributed to the development of pulmonary LCH.
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PMID:Pulmonary Langerhans cell histiocytosis associated with lingual carcinoma. 1546 72

The tobacco smoke intake by the smoker or by the non-smoker is a toxic product and a risk factor for diseases as acne in teenagers, respiratory and non respiratory infection, or histiocytosis X. The four main diseases are: chronic obstructive pulmonary disease, which is more 80% related to tobacco; cancer (lung carcinoma, pharyngeal, laryngeal and oesophageal carcinoma, kidney, bladder carcinoma); cardiovascular diseases (myocardial infarction, cerebral stoke and sudden cardiac death); adverse consequences of smoking related to pregnancy.
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PMID:[Tobacco-related diseases]. 1565 11

Erdheim-Chester disease (ECD) is a rare xanthomatous non-Langerhans cell histiocytosis which involves the marrow space of the long bones. Extraosseous sites most commonly affected include the eyes, lungs, pituitary glands, and kidneys. We report the case of a 49-year-old woman who presented with palpable breast nodules, followed by progressive soft tissue and subcutaneous disease, and involvement of the long bones, dysarthria, and dysphagia. The histopathologic features and skeletal radiography findings are consistent with ECD. This case represents an unusual presentation, which led to delayed diagnosis, as ECD of the breast has been rarely reported. ECD should be considered in the differential diagnosis of histiocytoid breast lesions, including fat necrosis and histiocytoid invasive mammary carcinoma.
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PMID:Erdheim-Chester disease of the breast: a case report and review of the literature. 1629 93

In the following, we describe the very rare case of Langerhans cell sarcoma (LCS) in the lung. Throughout the medical literature, only a few cases have been published, and, to the best of our knowledge, this is the first case to be reported in Germany. The patient was an 81-year-old man who showed symptoms such as chronic cough and weight loss. Clinical examination including needle biopsy indicated a high possibility of carcinoma in the right lung and in the mediastinum; however, the final histopathological diagnosis after immunohistochemistry gave evidence of LCS. LCS is a neoplastic proliferation of Langerhans cells with malignant cytological features exhibiting a very aggressive behaviour. LCS can be distinguished from other carcinomas, lymphomas and sarcomas by the typical morphological features of Langerhans cells and the immunophenotype with a consistent expression of S-100 protein and CD1a. In contrast to Langerhans cell histiocytosis, the LCS consists of Langerhans cells with high atypia and a very high mitotic rate.
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PMID:Pulmonary manifestation of a Langerhans cell sarcoma: case report and review of the literature. 1632 50

A total of 135 pediatric head and neck tumors diagnosed in our institute were reviewed with a view to elucidate the overall cytological patterns and analyze the important cytological features. Ninety-four tumors (69.6%) were aspirated for a primary diagnosis, and in 41 (30.4%) cases, fine-needle aspiration cytology was performed to document relapse, recurrence or a metastasis. Among the 94 tumors aspirated for a primary diagnosis, 66 cases (70.2%) were accurately diagnosed, in 22 cases (23.4%) a broad working diagnosis was offered, and 6 cases (6.4%) were misdiagnosed. The accuracy rate was higher (79.3%) when relapse-recurrent and metastatic tumors were included. The smears were broadly divided into six patterns, viz. round cell, epithelial, anaplastic, giant cell, mixed inflammatory, and spindle cell patterns. The round cell pattern was the most frequent one encountered in this group. The cytological features that stood the test of variability were lymphoglandular bodies and a noncohesive cell population in hematolymphoid malignancies, pale chromatin and cytoplasmic vacuoles in primitive neuroectodermal tumor/Ewing's sarcoma (PNET/ES), neuropil and rosettes in neuroblastoma, and plasmacytoid rhabdomyoblasts in rhabdomyosarcoma. A fairly good accuracy was seen in the diagnosis of metastatic undifferentiated carcinoma and anaplastic lymphoma, but the giant-cell and spindle-cell tumors continued to pose a problem in diagnosis. Ancillary techniques such as immunocytochemistry and electron microscopy applied in limited cases helped evaluate Langerhans cell histiocytosis, alveolar rhabdomyosarcoma, and the PNET/ES family of tumors.
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PMID:Cytological evaluation of head and neck tumors in children--a pattern analysis. 1668 Jul 71


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