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)

We report the results of a prospective registration of the prevalence of interstitial lung diseases by 20 centres of respiratory medicine in Flanders from January 1992 till July 1996. The 20 centres registered a total of 362 cases. Sarcoidosis (31% with inclusion of stage I, or 22% without stage I), idiopathic pulmonary fibrosis (20%), hypersensitivity pneumonitis (13%) and unclassified forms of interstitial lung diseases (9%) were the most often diagnosed diseases. The mean age of the patients was 52 +/- 17 (SD) years, but ranged from 32 +/- 10 years in histiocytosis X to 70 +/- 13 years in drug induced interstitial lung diseases. The male to female ratio was 1.3 but ranged from 2.3 in interstitial lung diseases due to inhalation of inorganic materials to 0.8 in interstitial lung diseases due to connective tissue disease. The diagnosis was considered certain in 60%, probable in 30% and possible in 10%. In 50% of the cases the diagnosis was proven by biopsy and the most often used biopsy technique was transbronchiolo-alveolar biopsy (40% of biopsies) followed by open lung biopsy (32%). At diagnosis vital capacity was 82 +/- 22% pred. (ranging from 92 +/- 19% in sarcoidosis to 71 +/- 24% in idiopathic pulmonary fibrosis) and CO-diffusing capacity was 77 +/- 19% pred. (ranging from 77 +/- 19% in sarcoidosis to 41 +/- 13% in drug induced pneumonitis). The regional frequency of interstitial lung diseases in Flanders varied widely and was not proportional to the regional distribution of the 20 contributing centres. In conclusion this registration provides interesting information on the occurrence and presentation of the different forms of interstitial lung diseases, although it may still underestimate the real prevalence and incidence of interstitial lung diseases.
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PMID:Registration of interstitial lung diseases by 20 centres of respiratory medicine in Flanders. 1148 13

The aim of this pictorial essay is to illustrate the distribution of normal findings and various lung diseases on coronal reconstructions as compared with cross-sectional high-resolution CT images. The volumetric CT images were obtained at 120 kVp, 200 mA/rotation, pitch 6:1, and a high-spatial-frequency reconstruction algorithm. The scans were performed using 2.5-mm collimation and reconstructed at 1.25-mm intervals. Coronal reconstructions were obtained at 2.5-mm slice thickness and 2.5-mm intervals. The pictorial includes images of normal anatomy, emphysema, idiopathic pulmonary fibrosis, Langerhans cell histiocytosis, sarcoidosis, bronchiolitis obliterans organizing pneumonia, desquamative interstitial pneumonia, nonspecific interstitial pneumonia, lymphocytic interstitial pneumonia, pulmonary edema, tuberculosis, cytomegalovirus pneumonia, bronchiectasis, and panbronchiolitis.
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PMID:Multidetector spiral high-resolution computed tomography of the lungs: distribution of findings on coronal image reconstructions. 1236 68

Bronchiolar abnormalities are relatively common and occur in a variety of clinical settings. Various histopathologic patterns of bronchiolar injury have been described and have led to confusing nomenclature with redundant and overlapping terms. Some histopathologic patterns of bronchiolar disease may be relatively unique to a specific clinical context but others are nonspecific with respect to either etiology or pathogenesis. Herein, we present a scheme separating (1) those disorders in which the bronchiolar disease is the predominant abnormality (primary bronchiolar disorders) from (2) parenchymal disorders with prominent bronchiolar involvement and (3) bronchiolar involvement in large airway diseases. Primary bronchiolar disorders include constrictive bronchiolitis (obliterative bronchiolitis, bronchiolitis obliterans), acute bronchiolitis, diffuse panbronchiolitis, respiratory bronchiolitis, mineral dust airway disease, follicular bronchiolitis, and a few other rare variants. Prominent bronchiolar involvement may be seen in several interstitial lung diseases, including hypersensitivity pneumonitis, respiratory bronchiolitis-associated interstitial lung disease, cryptogenic organizing pneumonia (idiopathic bronchiolitis obliterans organizing pneumonia), and pulmonary Langerhans' cell histiocytosis. Large airway diseases that commonly involve bronchioles include bronchiectasis, asthma, and chronic obstructive pulmonary disease. The clinical relevance of a bronchiolar lesion is best determined by identifying the underlying histopathologic pattern and assessing the correlative clinico-physiologic-radiologic context.
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PMID:Bronchiolar disorders. 1464 23

Simultaneous bilateral spontaneous pneumothorax (SBSP) is a very rare condition, mainly seen in patients with underlying lung disease. Up to now, there are 65 patients who have been published. Twelve consecutive patients who presented with SBSP as definitive diagnosis were recruited for this study. They represented 1 % of all patients with spontaneous pneumothorax. All patients had immediate bilateral chest tubes on admission. Five of the 12 patients (42%) had no underlying lung disease. In 7 patients, SBSP was secondary to pulmonary metastases, histiocytosis X, undefined interstitial pulmonary disease, tuberculosis, pneumonia and chronic obstructive pulmonary disease. None of the patients died during hospitalization. Eleven patients were treated with chemical pleurodesis, whereas thoracotomy and pleurectomy were necessary in 7 patients. Reexpansion of the lungs was achieved in all patients. Immediate bilateral chest tube insertion and pleurodesis are of major importance in the treatment of SBSP although a subset of patients needed surgical pleurectomy. Combination of these treatments provides successful and uneventful treatment of the disease.
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PMID:Simultaneous bilateral spontaneous pneumothorax report of 12 cases and review of the literature. 1557 Oct 26

Pulmonary lesions encountered by the pathologist in which histiocytes are the dominant finding histologically are reviewed. Lesions discussed include neoplasms of histiocytes and nonneoplastic processes. The nonneoplastic processes are divided into those that present as nodular histiocytic proliferations in the lung, those that present as diffuse proliferations of histiocytes in the lung, and those with a mixed pattern. Entities discussed include pulmonary Langerhans' cell histiocytosis, pneumonoconioses, infections, diffuse panbronchiolitis, crystal storing histiocytosis, respiratory bronchiolitis, alveolar hemorrhage, eosinophilic pneumonia, obstructive pneumonia, exogenous lipoid pneumonia, some drug reactions, and some metabolic/storage diseases. Entities of uncertain histogenesis, including Rosai-Dorfman disease and Erdheim-Chester disease, are also discussed. Qualitative features of the histiocytes are addressed, including the presence of foreign dust, hemosiderin, foamy change, and histiocytes showing features of Langerhans' cells.
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PMID:Histiocytic lesions and proliferations in the lung. 1788

Cigarette smoke, a toxic collection of more than 4000 chemicals generated from combustion of tobacco plant leaves, is known to cause several respiratory ailments, including chronic bronchitis, emphysema and lung cancer, and is associated with an increase in respiratory infections. In addition, cigarette smoking is considered a principal aetiological factor responsible for the development of certain diffuse interstitial and bronchiolar lung diseases, namely respiratory bronchiolitis-interstitial lung disease (RB-ILD), desquamative interstitial pneumonia (DIP) and adult pulmonary Langerhans' cell histiocytosis (PLCH). Although not exclusively seen in cigarette smokers, substantial clinical and epidemiological data support a central role for smoking as the primary causative agent of most RB-ILD, DIP and PLCH. Additional evidence in support of cigarette smoke as a primary aetiological agent in RB-ILD, DIP and PLCH is the observation that smoking cessation may lead to disease improvement, while recurrence of these disorders has been observed to occur in the transplanted lung upon re-exposure to tobacco smoke. Furthermore, histopathological changes of respiratory bronchiolitis, DIP and PLCH (with or without co-existent emphysema) may be found on lung biopsy in the same individual, implicating smoking as a common inciting agent of these diverse lesions. Recent studies also suggest a role for cigarette smoking as a potential co-factor in the development of acute eosinophilic pneumonia, usual interstitial pneumonia and rheumatoid arthritis-associated interstitial lung disease. In the current review, we propose a novel classification that takes into account the complex relationship between cigarette smoking and diffuse lung diseases. Investigation on the role of smoking as a potential causative factor or modifier of these diverse diffuse lung diseases is important, as smoking cessation utilizing state-of-the-art tobacco cessation efforts should be a central part of therapy, while pharmacotherapy with corticosteroids or other immune modifying agents should be reserved for selected patients.
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PMID:Cigarette smoking and diffuse lung disease. 1862 8

Allogeneic stem cell transplant is curative for haemophagocytic lymphohistiocytosis (HLH) and refractory Langerhans cell histiocytosis (LCH). However, patients frequently have significant pre-transplant morbidity and there is high TRM. Because HLH is caused by immune dysregulation, we surmised that a reduced-intensity conditioned (RIC) regimen might be sufficient for cure, while decreasing the TRM. In 2006, we reported the outcome of 12 patients treated with RIC SCT from a matched family/unrelated or haploidentical donor. Here we discuss the update of these patients, including a total of 25 patients treated with RIC SCT for HLH and three for LCH. Twenty-one of the twenty-five patients with HLH (84%) are alive and well with remission at a median of 36 months from SCT. Mortality included pneumonitis (n=3) and hepatic rupture (n=1). All three patients treated with RIC SCT for LCH remain alive and in remission at a median of 5.1 years from SCT. Seven of twenty-four survivors (one with LCH) have mixed chimerism but remain disease-free. These data are supported by other groups including 100% survival in seven patients with HLH and 78% survival of nine patients with LCH. In summary, RIC compares favourably with conventional SCT with long-term disease control in surviving patients with both HLH and LCL, despite a significant incidence of mixed chimerism.
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PMID:The use of reduced-intensity stem cell transplantation in haemophagocytic lymphohistiocytosis and Langerhans cell histiocytosis. 1897 44

Cigarette smoking has been associated with several diffuse lung diseases in which both bronchiolar and interstitial lung inflammation appear to result from chronic tobacco smoke inhalation. These diseases occur primarily in relatively young adult smokers and include desquamative interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, and pulmonary Langerhans cell histiocytosis. Although these diseases are associated with characteristic histopathologic and radiological features, there is significant overlap among these diseases, and some smokers may exhibit features indicative of the broad spectrum of smoking-related interstitial and bronchiolar lung injury patterns. Cigarette smoking has also been associated with acute eosinophilic pneumonia, and it increases the risk of developing other fibrotic interstitial lung diseases such as idiopathic pulmonary fibrosis and rheumatoid arthritis-associated interstitial lung disease. Although relatively uncommon, these smoking-related diffuse lung diseases should be recognized by clinicians as an important subset in the spectrum of interstitial lung diseases in which smoking cessation forms a central part of disease management. With the exception of acute eosinophilic pneumonia, which responds well to corticosteroid therapy, the role of corticosteroid and other immune-suppressive treatments in the management of smoking-related interstitial lung diseases is not entirely clear and is probably of limited utility, particularly in the absence of smoking cessation.
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PMID:Tobacco smoke-related diffuse lung diseases. 1922 62

We describe a rare case of secondary malignant fibrous histiocytoma (MFH) following Langerhans cell histiocytosis (LCH). A 23-year-old Japanese male exhibited systemic lymphadenopathy, multiple lung tumors, and osteolytic changes in bilateral iliac bones in 1989. A biopsy specimen from the left iliac bone revealed an infiltration of S-100 protein-positive histiocyte-like cells intermingled with eosinophils, which confirmed the diagnosis of eosinophilic granuloma, a type of LCH. Although the patient was treated with prednisolone initially, the disease did not respond well and progressed gradually over time. The patient subsequently received multiple courses of chemotherapy and immunosuppressive therapy with many kinds of anticancer agents for 6 years. He also received radiotherapy totaling 136.8 Gy for lung tumors and osteolytic lesions of the pelvis. In 1997, because of the LCH refractoriness, biopsy was performed again from the right inguinal lymph node. Microscopic examinations demonstrated a mixture of spindle-shaped cells and histiocyte-like cells, which appeared to be in a storiform pattern. The tumor cells were immunohistologically positive for CD68 and vimentin, but negative for CD1a and S-100 protein. Therefore, the patient was diagnosed with MFH. Although chemotherapy was continued, the patient died of pneumonia during the neutropenic period following chemotherapy. Autopsy revealed systemic invasion of MFH and dissemination of mucormycosis. LCH was not detected histologically in any tissues.
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PMID:Secondary malignant fibrous histiocytoma following refractory langerhans cell histiocytosis. 1947 15

The association of multiorgan histiocytosis after acute lymphoblastic leukemias is very rare as most cases are localized forms of Langerhans cell histiocytosis (LCH). We report on an 18-year-old man diagnosed with B-cell acute lymphoblastic leukemia (B-ALL) with p16 deletion (9p21). He was treated with induction chemotherapy using the Spanish PETHEMA group protocol and achieved complete remission. Three months after the diagnosis of B-ALL, he developed a severe multiorgan histiocytosis that is clinically suggestive of LCH but lacked typical immunohistochemical features of LCH and indeterminate cell histiocytosis: CD1a was strongly positive, CD68 and S-100 protein were moderately positive, and langerin was negative. The drugs of the first-line treatment recommended for LCH had been part of the chemotherapy of B-ALL that the patient had received. Therefore, we prescribed the second-line treatment for LCH (cytarabine and 2'-chlorodeoxyadenosine), and he achieved partial remission. The patient died during the aplasia induced by the third cycle of chemotherapy from pneumonia. We could not demonstrate the transdifferentiation of tumoral lymphocytes into histiocytes, using p16 deletion (9p21) as a marker, because these cells did not share the mutation. Neither could we study immunoglobulin-H rearrangement as we had exhausted all the tissue samples. In the medical literature, there are a few reported cases of T-cell acute lymphoblastic leukemia followed by disseminated LCH and just 1 case of B-ALL followed by localized LCH affecting the bones. Therefore, our patient may be the first published case of B-ALL followed by histiocytosis, which had 2 singularities: it was multiorgan and the immunohistochemistry was not typical of LCH.
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PMID:Multiorgan histiocytosis after B-cell acute lymphoblastic leukemia. 2128 61


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