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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two cases of leptomeningo-cerebro(-spinal) sarcoidosis which were not clinically diagnosed in two women (22 and 33 years old) are described. The two women died because of this disease, 8 months, and 1 1/2 years, resp., after the first manifestation. In the first case there was a largely isolated local affection of the hypothalamus and neurohypophysis. In the second observation, beside an involvement of the bifurcate lymph nodes and of the lungs, it was only the central nervous system that showed massive diffuse changes partially due to a granulomatous angiitis of cerebral and visceral vessels. On the basis of pathohistology, the differential diagnosis to the histiocytosis X for case 1 and to the granulomatous giant cell angiitis for case 2 are discussed.
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PMID:[Cerebrospinal sarcoidosis (with reference to 2 personal cases)]. 90 97

We report the results of a morphological analysis of 60 pulmonary biopsies gathered from a multi center study, organised by the clinico-pathological research group on Wegener's Disease under the auspices of the French Language Society of Thoracic Medicine. Forty of the sixty cases analysed were retained after indexing the histological aspects in order to specify their diagnostic value. Two groups of lesions were distinguished, which had different significance. Group A: These include the three major diagnostic criteria, which reinforce one another as they associate: 1) The polymorphoneutrophil microabscesses with limited central necrosis or an extended necrosis like the contours of a relief map. 2) An angiitis (arteries, veins, capillaries) with eccentric focal parietal crescent-shaped microabscesses. 3) Polymorphous granulomas with giant cells. Group B: In this group are the minor morphological observations (table II) of a lesser value and significance. 1) Acute or chronic lesions with alveolar haemorrhage, endogenous lipid pneumonia, xanthomatous granulomas, an organising pneumonia with an alveolitis. 2) Bronchial lesions: Bronchitis and necrotising bronchiolitis, which is more rarely follicular. 3) Sero-fibrinous or infiltrative neutrophil pleural lesions with focal microabscesses, elastolysis and elastophagia with giant cells in the elastic lamina. Thirteen cases presented with misleading lesions, which was a possible source of diagnostic error and led to a discussion of several associated disorders (Goodpasture's syndrome, and collagen disorder syndrome) or there may be systemic angiitis (Giant cell or lymphocytic) or also systemic or tissue eosinophilia (Churg-Strauss syndrome, bronchocentric granulomatosis) or necrotising bronchitis (atrophic polychondritis) or other forms of nodular interstitial fibrosis, such as histiocytosis X. We would like to stress the great polymorphic variation of the lesions and the difficulties which confront pathologists in the diagnosis of Wegener's Disease, above all when it is localised to the lung. There is value in finding at least one major diagnostic criteria which is associated with a minor criteria and with the help of the C.ANCA levels may lead to a narrow clinicopathological correlation and allows for a fairly precise approach to the diagnosis and identification of early or unusual lesions and thus to the early treatment of patients before irreversible renal failure appears.
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PMID:[Pulmonary lesions in Wegener's disease. Report of the French Anatomo-clinical Research Group. Study of 40 pulmonary biopsies]. 150 87

The accumulated experience and the literature data allow for the separation of a group of granulomatous pulmonary diseases among pulmonary diseases of different etiology. Etiologically heterogeneous granulomatous diseases are united by the general signs: granuloma development, immunologic disturbances mainly within the cellular system and mediators as well as systemic vascular affection in the form of vasculitis. In our opinion, granulomatous diseases include disseminated tuberculosis, sarcoidosis of respiratory organs, exogenic and idiopathic fibrous alveolitis, Wegener's granulomatosis, histiocytosis X, primary hemosiderosis, Goodpasture's syndrome and some other rare diseases. Granulomatous diseases are diagnosed on the basis of the ++clinico-roentgenologic findings with an obligatory cytological and histological study of the bioptic specimen; immunologic diseases are diagnosed proceeding from the study of the immunologic status and detection of specific antibodies; of great significance for the diagnosis of pulmonary granuloma caused by infectious pathogens are microbiologic studies which provide for the detection of microorganisms and fungi. Study of the clinicoroentgenologic and laboratory data made it possible to distinguish a number of features typical for each disease and to unite them into diagnostic symptom complexes. Despite the different course of granulomatous pulmonary diseases they may end in recovery and granuloma resolution, development of lung fibrosis in a chronic course and in certain diseases in lung tissue destruction with cavity formation (tuberculosis, Wegener's granulomatosis). A fatal outcome may also ensue due to an acute or chronic course of the diseases.
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PMID:[Diagnosis of granulomatous lung diseases]. 187 Oct 95

The interstitial lung diseases are comprised of a group of pulmonary disorders characterized clinically by diffuse infiltrates on the chest radiograph and histologically by distortion of the gas exchanging portion of the lung. The physiologic correlates are restriction of lung volumes and impaired oxygenation. The term "interstitial" when applied to these diseases is actually a misnomer because it implies that the inflammatory process is limited specifically to the area between the alveolar epithelial and capillary endothelial basement membranes. The diseases currently grouped as "interstitial" also frequently involve the alveolar epithelium, alveolar space, pulmonary microvasculature, and less commonly, the respiratory bronchioles, larger airways, and even the pleura. The enormous differential diagnosis of interstitial lung disease can be made manageable by understanding that pneumoconiosis, drug-induced disease, and hypersensitivity pneumonitis account for over 80% of the responsible entities and can usually be identified from the patient's history. The nine remaining diseases/disease categories include: sarcoidosis, idiopathic pulmonary fibrosis, bronchiolitis obliterans-organizing pneumonia, histiocytosis X, chronic eosinophilic pneumonia, collagen vascular disease-associated interstitial lung disease, granulomatous vasculitis (Wegener's granulomatosis, Churg-Strauss syndrome, lymphomatoid granulomatosis), Goodpasture's syndrome, and pulmonary alveolar proteinosis. The diagnosis of a specific interstitial lung disease can be made via various means including the patient's history, specific serologies, bronchoalveolar lavage, transbronchial biopsy, and biopsy of extrathoracic tissues or open lung biopsy. A directed diagnostic approach can be formulated based on an understanding of these techniques and a thorough knowledge of the clinical presentations and specific diagnostic criteria for each of the major diseases. This monograph will serve as a guide for the clinician to use in evaluating and treating patients with interstitial lung disease. We begin by reviewing the clinical presentation, diagnostic criteria, and management of specific interstitial lung diseases excluding pulmonary infection, neoplasm, and sarcoidosis. Pneumoconiosis and drug-induced syndromes are not discussed in detail, but the agents responsible and pertinent exposures are presented in tabular form in the discussion of the general diagnostic approach.
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PMID:Interstitial lung disease. 199 45

Worldwide almost no epidemiologic data are available on the prevalence or incidence of interstitial lung diseases (ILD) in the general population. Therefore, a registration programme of ILD-prevalence was organised by the VRGT (Vereniging voor Respiratoire Gezondheidszorg en Tuberculosebestrijding), among about 100 Flemish pneumologists since 1990. Most categories of the classification by Crystal et al. (1) were included and the diagnostic criteria (histology, laboratory tests, clinic, radiology) were registered. The present paper presents the results of 1992-1994: twenty pneumologists had forwarded the summary files of 237 patients to the central office in 1992 (n = 68), 1993 (n = 90) and 1994 (n = 79). The diagnoses that were most frequently made were: sarcoidosis in 27%, idiopathic pulmonary fibrosis in 20%, hypersensitivity pneumonitis in 14% (of which 68% by birds) and collagen-vascular disease in 10% (of which 54% in rheumatoid arthritis). Less frequent causes were eosinophilic pneumonia (4%), inhalation of inorganic material (4%, anthracosilicosis being excluded), histiocytosis X (3%), drugs (3%), angiitis and granulomatosis (2%), pulmonary hemosiderosis (1%), lymphocytic infiltrative lung disease (1%) and lymphangioleiomyomatosis (1%). The order of relative frequencies of the different categories of diseases was the same in the 3 registration years. In 9% of the patients the diagnosis was confined to "undefined fibrosis". The diagnosis was confirmed by histology in 63% of the cases. The overall male-female ratio was nearly one, with, however, a male preponderance in hypersensitivity pneumonitis (22/12), UIP(8/3) and "undefined fibrosis" (14/7).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Epidemiology of interstitial lung disease (ILD) in flanders: registration by pneumologists in 1992-1994. Working group on ILD, VRGT. Vereniging voor Respiratoire Gezondheidszorg en Tuberculosebestrijding. 853 25

Langerhans' cell histiocytosis (LCH) is a rare condition of children and young adults in which Langerhans' cells proliferate. The clinical spectrum ranges from solitary or few focal lesions to multisystem involvement mimicking vasculitis or hematological malignancy. Focal bone lesions, known as eosinophilic granulomas, are the most common manifestations. Eosinophilic granuloma usually presents with a variable combination of pain, swelling, fracture, and fever. Facial bone involvement may manifest as an ear discharge, hearing loss, or exophthalmos. Nerve root pain is rarely reported, even in patients with lesions in the axial skeleton. We report four cases of nerve root pain caused by LCH. Two male patients aged 25 and 34 years, respectively, presented with truncated femoral neuralgia related to acetabular granulomas. A 25-year-old woman with involvement of the L5 vertebral body and a 41-year-old man with a sacral lesion presented with sciatica.
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PMID:Radiculopathy as a manifestation of Langerhans' cell histiocytosis. 1734 85

Intra-axial pseudotumors in the central nervous system often mimic malignant brain tumors and cause difficulty in diagnosis and treatment. The present study investigates their radiologic and histological features to elucidate diagnostic clues. Six cases were included in the study, one man and five women, ranging in age from 44 to 87 years (mean age, 61 years). Histologically, three cases had demyelination, and one case each had abscess, angiitis, and non-Langerhans cell histiocytosis. All cases were evaluated radiologically on MRI, most of them by thallim-201 single photon emission tomography ((201)Tl-SPECT). These cases were examined using H&E, special stains, and immunohistochemical studies with a variety of antibodies. MRI demonstrated perifocal edema and ring-like or solid enhancement, mimicking the malignant tumors. Diffusion-weighted MRI showed a hypo-iso-intensity with a hyperintensity on the apparent diffusion coefficient. A (201)Tl-SPECT study revealed no uptake. Although there were various kinds of pathology, inflammatory cells were observed, associated with vascular proliferation and reactive astrocytosis. In addition, some cases showed demyelinating or destructive changes. These results suggested that intra-axial pseudotumors in the central nervous system contain various kinds of pathology, and detailed clinicopathological analysis is important from the point of view of differential diagnosis.
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PMID:Intra-axial pseudotumors in the central nervous system: clinicopathological analysis. 2104 8

Pulmonary involvement in systemic diseases is common, but the radiographic appearance of early-stage pulmonary changes is often subtle. Computed tomography (CT) has higher sensitivity and specificity than radiography, and high-resolution CT is the method of choice for accurate assessment of diffuse parenchymal lung disease. Even with reductions in the peak voltage and tube charge to minimize the exposure of pediatric patients to radiation, CT performed with a meticulous acquisition technique can provide detailed information. In some cases, high-resolution CT may depict clinically silent lung lesions. The information provided by CT is invaluable for planning therapy in pediatric patients with pulmonary involvement in connective tissue disease (eg, juvenile rheumatoid arthritis, dermatomyositis, systemic sclerosis, systemic lupus erythematosus, or mixed connective tissue disease), vasculitis, a primary or acquired immune deficiency disorder, immotile cilia syndrome, cystic fibrosis, or Langerhans cell histiocytosis.
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PMID:Thoracic findings of systemic diseases at high-resolution CT in children. 2141 92

Frosted branch angiitis is a rare vascular reaction believed to be a nonspecific immune response to an infective, neoplastic, or idiopathic insult. The clinical presentation is characteristic and typically affects children and younger adults, and the prognosis is good. We report a case of frosted branch angiitis during immune recovery in a 2-year-old boy with Langerhans cell histiocytosis on systemic immunosuppressive therapy.
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PMID:A case of frosted branch angiitis in an immunocompromised child. 2572 93

Here, the authors present the first documented case of a patient developing central nervous system (CNS) vasculitis secondary to Langerhans cell histiocytosis (LCH) ultimately leading to stroke. LCH is a rare histiocytic disorder affecting males and females equally and typically presents in pediatric patients with a median age of 30 months. Presentation of the disease can be single-site or multisystem; and, classification of treatment is further demarcated by high risk and low risk depending on the organ systems involved. Treatment of LCH typically involves vinblastine and prednisone, as well as salvage treatment as needed.
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PMID:Langerhans Cell Histiocytosis Causing Central Nervous System Vasculitis Leading to Stroke in a Two-year-old Boy: A Case Report. 3093 49


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