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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 17 year old female was seen because of a non-productive cough and one episode of blood-tinged sputum. A computerized tomography (CT) scan of the chest showed multiple small cystic lucencies in the upper lobes. The mediastinal CT window revealed concurrent mediastinal lymphadenopathy. An open lung biopsy showed Langerhans' cell histiocytosis (LCH). The significance of nodal involvement in LCH of the lung is not known, but raises the possibility of regional LCH and warrants a further search for bone or visceral involvement as well as closer follow-up and monitoring of patients.
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PMID:Langerhans' cell histiocytosis of the lung in association with mediastinal lymphadenopathy. 856 38

Langerhans' cell histiocytosis (LCH) is a clonal proliferation of Langerhans cells (LC) showing histologically an abundant reactive infiltrate composed of macrophages and lymphocytes, as well as eosinophilic and neutrophilic granulocytes. Rosai-Dorfman disease (RDD) shows a sinusoidal accumulation of large histiocytic cells with an immunophenotype similar to LC of LCH. The histological picture of LCH is reminiscent of an inflammatory disorder and LC may produce cytokines and are influenced by these soluble factors. This study set out to establish the monokine expression pattern in LCH in comparison with those of RDD; dermatopathic lymphadenopathy, which also shows a proliferation of S100-positive dendritic cells; and LC in normal skin specimens. Isotopic in situ hybridization was used for the detection of transcripts of tumour necrosis factor-alpha (TNF-alpha), interleukin-6 (IL-6), and IL-1 beta, in some cases combined with immunohistology for the S100 protein or CD68. In all 11 tissue samples from eight patients, LC of LCH expressed TNF-alpha; in two cases IL-1 beta transcripts were additionally noted in some LC, whereas IL-6 was found in reactive cells. Large histiocytic cells of RDD expressed all three monokines, whereas minimal or no expression of these cytokines could be detected in interdigitating reticulum cells in dermatopathic lymphadenopathy. In two out of five normal skin samples, only TNF-alpha specific signals were observed in LC. These data suggest that histologically different lesions of the histiocytic/dendritic cell system display distinct cytokine profiles. The expression of monokines, which have been demonstrated to influence various functions of epidermal LC, may play a role in the pathogenesis of LCH. Systemic symptoms in RDD may be related to enhanced production of monokines in these lesions.
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PMID:Monokine expression in Langerhans' cell histiocytosis and sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) 912 Jul 35

A 48-year-old female with a history of accentuated dyspnea, pleural thickening in anteromedial portion with left patchy parenchymal shadowing invading adjoining parasternal structure of the rib cage, presented 13 months later marked superclavicular, anterior mediastinic and parahilar left lymphadenopathy. Open surgical biopsies on the pleural lesion invading the hypodermic tissues of parasternal region showed morphological and immunocytochemical patterns of Langerhans' cell histiocytosis (LCH). 13 months later the superclavicular lymph node biopsy diagnosed Hodgkin's lymphoma (HD), mixed cellularity type II, stage AE. On a total of 29 cases with association of LCH and HD, the described case in the second case that shows morphologically demonstrated LCh with subsequent development of HD. It is postulated that the development of HD in a patient with LCH, might represent malignant evolution of this hyperplastic process.
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PMID:Langerhans' cell granulomatosis and Hodgkin's lymphoma. Report of a case. 870 Mar 50

A 28-year-old woman was admitted to our hospital with the chief complaint of progressive gingival swelling with easy bleeding and loosening of teeth for about two years. The liver and spleen were not enlarged and there were no lymphadenopathy. The hemogram was normal. The skull X-ray showed floating teeth. CT scan of face showed destructive bony lesions over maxilla and left mandible with adjacent soft tissue swelling. Pathologic examination of the gingiva revealed that the oral mucosal tissue was heavily infiltrated with histiocyte-like Langerhans' cells. The Langerhans' cells showed positive immunostain for S-100 protein. Under electron microscope, ultrastructure of the Langerhans' cells revealed typical intracytoplasmic tennnis racquet-shaped structure with a central zipper-like striation (Birbeck's granules). Langerhans' cell histiocytosis was diagnosed. She received rdiotherapy with a total dose of 1,000 cGy in fractions and oral chemotherapeutic drugs. Painful gingival swelling subsided gradually. She was followed at our OPD for the past 10 months and there was no evidence of local recurrence. Langerhans' cell histiocytosis is a rare disease. The relevant literature about its distinct pathologic features, clinical course and treatment is reviewed.
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PMID:Langerhans' cell histiocytosis (histiocytosis-X) in a women with typical ultrastructure of Birbeck's granule: a case report. 899 34

The health risks associated with cigarette smoking are well known. Cigarette smoking is the most important causative factor in the development of bronchogenic carcinoma. Pulmonary diseases such as chronic bronchitis, centrilobular and panacinar emphysema, respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), and pulmonary Langerhans cell histiocytosis are also related to cigarette smoking. In adenocarcinoma and squamous cell carcinoma, the most common manifestation at computed tomography (CT) is a solitary pulmonary nodule; in small cell carcinoma, hilar and mediastinal adenopathy secondary to metastases; and in large cell carcinoma, a mass with central necrosis or cavitation in the lung periphery. For chronic bronchitis, the most common CT finding is bronchial wall thickening, but this finding is nonspecific. Emphysema, both centrilobular and panacinar associated with alpha-1-antitrypsin deficiency, usually manifests as areas of decreased attenuation and may involve bullous changes. However, centrilobular emphysema more commonly involves the upper lungs, whereas panacinar emphysema more commonly involves the lower lungs. Most patients with RB-ILD have normal high-resolution CT scans; however, abnormalities may be present, the most common of which are areas of ground-glass attenuation. CT appearance of pulmonary Langerhans cell histiocytosis varies depending on the disease stage: In the early stage, typical CT findings include multiple nodules, usually accompanied by cystic spaces with thin, well-defined walls. As the disease progresses, the cystic spaces become more numerous and the number of nodules decreases.
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PMID:Cigarette smoking: CT and pathologic findings of associated pulmonary diseases. 908 79

Two cases of congenital systemic Langerhans cell histiocytosis (LCH), diagnosed and treated in our department from June 1995 until May 1996, are described. The cases concern two neonates (one female and one male) born with necrotic lesions and skin nodules. The diagnosis was confirmed by skin biopsy which showed diffuse infiltration by CD1 antigen and S-100 protein positive histiocytes. The babies didn't present with anemia, hepatosplenomegaly or lymphadenopathy. Hepatic and renal function were normal. In both infants skeletal survey showed no lytic lesions but chest X-rays and high resolution computerized tomography (HRCT) scan revealed diffuse mottling of both lung fields. Bone marrow aspiration showed the presence of histiocytes in percentages of 6% and 10%, respectively. Both babies were treated with prednisolone 1 mg/kg body weight for three months. The first child who is 20 months old, is now well with resolution of skin and pulmonary lesions occurring within one month of the initiation of steroids, while the second, who presented spectacular resolution of skin lesions within the first three weeks of therapy, is also in excellent condition five months after completion of treatment. We conclude that congenital LCH has to be suspected in neonates with persisting skin lesions. If the disease is systemic but without organ dysfunction, treatment with steroids may be beneficial.
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PMID:Congenital systemic Langerhans cell histiocytosis (report of two cases). 940 65

Cat-scratch disease is a self-limited condition commonly causing a benign chronic lymphadenopathy in children. Osteolytic lesions are a rare complication, but have been previously reported. We report a case of a solitary osteolytic lesion of the skull whose clinical, radiographic and pathological features were initially interpreted as being consistent with Histiocytosis X. Subsequently, positive serological titers for Bartonella, a history of a cat-scratch antecedent to the onset of clinical symptoms and review of the original histopathology confirmed the diagnosis of cat-scratch disease. We reviewed the English language literature on osteolytic lesions associated with cat-scratch disease and compare the current case with those previously reported.
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PMID:Cat-scratch disease simulating Histiocytosis X. 963 88

Langerhans cell histiocytosis (LCH) has been thought to be a disorder of immune regulation, and increasingly, evidence showing that the tissue damage in LCH involves lymphokines and pro-inflammatory cytokines is reported. We detected human cytomegalovirus (HCMV)-DNA in LCH cells in the foci of LCH lesions by immunohistochemistry, in situ hybridization and PCR. HCMV was detected in the nuclei and/or cytoplasm of LCH cells in 9 of 27 LCH cases by immunostaining. HCMV was probably an early antigen. In situ hybridization revealed signals for HCMV-DNA only in the nuclei of LCH cells in 10 of the 27 LCH cases. PCR analysis was performed in 20 of the LCH cases, and HCMV-DNA was detected in 7 of these. All 7 positive cases were also positive for HCMV by ISH and IHC. These findings suggested that early phase infection or reactivation of HCMV occurred in the LCH lesions. HCMV infection may be accompanied by impaired cytokine production. Our study also suggested a relationship between HCMV infection and expression of TNFalpha. In tissues affected by LCH, dermatopathic lymphadenopathy or malignant fibrous histiocytoma and in normal tissues no signals for Epstein-Barr virus-RNA were detected. These findings suggest that in some cases LCH is associated with HCMV infection.
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PMID:Human cytomegalovirus infection in foci of Langerhans cell histiocytosis. 1007 Dec 44

We report a 74-year-old man who presented with a rash on the trunk showing clinical and histological features of non-Langerhans cell histiocytosis. Two years after presentation he developed weight loss, lymphadenopathy and hepatosplenomegaly; a diagnosis of lymphocyte-predominant Hodgkin's disease was made on lymph node biopsy. The cutaneous signs and lymphoma responded to chemotherapy. Taken in conjunction with previously published reports of associations between cutaneous histiocytoses and haematological malignancies, we recommend close observation of patients in whom a diagnosis of non-Langerhans cell histiocytosis is made.
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PMID:Non-Langerhans cell histiocytosis associated with lymphocyte-predominant Hodgkin's disease. 1056 22

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


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