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11/26. CT and MR imaging of CNS lymphomatoid granulomatosis.

    The clinical CT and MR imaging features of a pathologically confirmed case of lymphomatoid granulomatosis are presented. The disease was clinically confined to the central nervous system and the diagnosis was only made after brain biopsy had been performed. MR imaging revealed extensive non-confluent regions of white matter abnormality. Although uncommon, lymphomatoid granulomatosis should be included in the differential diagnosis of causes of periventricular and deep white matter lesions, even in the absence of pulmonary lesions. Specific therapy may produce clinical regression of disease.
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keywords = nervous system
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12/26. lymphomatoid granulomatosis of eyelid and brow with progression to lymphoma.

    lymphomatoid granulomatosis is an uncommon lymphoreticular disease that primarily involves the lungs, skin, and central nervous system and has a high mortality rate. This is the first report in the American literature of lymphomatoid granulomatosis with involvement of the skin of the eyelid and brow. After a protracted clinical course, the patient died of pulmonary complications.
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keywords = nervous system
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13/26. lymphomatoid granulomatosis: two cases with skin involvement.

    lymphomatoid granulomatosis is a systemic disease marked by a polymorphous cellular infiltrate that is both angiocentric and angiodestructive. The predominant organs of involvement are lungs, skin, central nervous system, and kidneys. I describe two cases of lymphomatoid granulomatosis in association with cutaneous manifestations, stressing to the dermatologist the importance of early recognition and diagnosis of this entity.
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keywords = nervous system
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14/26. Thoracic gallium uptake in patients with lymphomatoid granulomatosis.

    lymphomatoid granulomatosis (LG) is a rare condition with histological similarities to Wegener's granulomatosis and malignant lymphoma. Characteristically there is an angiocentric, angiodestructive lymphoreticular cell infiltrate. The lungs are usually affected, and, less frequently, the skin, nervous system, kidney, and bowel are involved. The prognosis is poor and frank lymphoma develops, in some cases terminally. The usual radiological appearance of the lungs consists of bilateral nodular lower zone opacities. The authors report two patients (siblings) with LG, and their gallium scans are presented. In each case there was a significant accumulation of gallium in the lungs at times of clinically active disease. The limited role of gallium imaging in this disease is discussed.
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ranking = 1
keywords = nervous system
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15/26. central nervous system lymphomatoid granulomatosis.

    lymphomatoid granulomatosis is a vasocentric lymphoreticular proliferative disease. Some patients go on to develop frank neoplasia. central nervous system involvement with lymphomatoid granulomatosis has been reported previously in the literature. We are presenting a childhood case evaluated with magnetic resonance imaging and x-ray computerized tomography who developed severe tissue destruction with pleomorphic cellular infiltrate and oligoclonal light chains. This report adds information regarding neuroimaging as well as immunopathology data relevant to the basic biology of this disease.
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ranking = 5
keywords = nervous system
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16/26. Cutaneous lesions of lymphomatoid granulomatosis.

    lymphomatoid granulomatosis is a rare multisystem disease involving the lungs, skin, and nervous system. The cutaneous disease may be the presenting complaint. A case with nodular and annular infiltrated lesions is reported to encourage the inclusion of lymphomatoid granulomatosis in the differential diagnosis of similar lesions. Prompt diagnosis and early long-term treatment with cyclophosphamide and prednisone can lead to prolonged remissions of this disease. If relapse occurs, prompt treatment with the same regimen may again induce a remission.
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keywords = nervous system
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17/26. lymphomatoid granulomatosis with ocular involvement.

    lymphomatoid granulomatosis is an angiocentric and angiodestructive lymphoproliferative disorder that involves multiple organs including the lung, skin, kidney and central nervous system. A case of lymphomatoid granulomatosis with ocular involvement is reported herein. Bilateral peripheral retinal vasculitis involving both arterioles and veins, with posterior uveitis, is the prominent clinical finding.
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ranking = 1
keywords = nervous system
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18/26. lymphomatoid granulomatosis. Case report from the thoracic services boston University Medical School.

    lymphomatoid granulomatosis is one of a group of pulmonary disorders characterized by necrotizing, aseptic granulomas, usually with angiitis. Multisystem dissemination is common especially to the skin and central nervous system. The diagnosis can be established only by histologic examination. Lymphomatous transformation apparently occurs in 10-20%. Treatment is generally unsatisfactory, although corticosteroids appear to be indicated initially. We present 4 patients, 3 of whom died relatively rapidly with progressive pulmonary lesions despite therapy with corticosteroids and cytotoxic agents. 1 of these patients developed a lymphoma. The fourth recovered after local excision. Pathologic diagnosis, classification, clinical and radiographic features and therapy are discussed.
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keywords = nervous system
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19/26. lymphomatoid granulomatosis involving the central nervous system: complication of a renal transplant with terminal monoclonal B-cell proliferation.

    A 33-year-old man, an immunosuppressed renal allograft recipient, developed fever of unknown etiology, multiple pulmonary densities, space-occupying lesions in the liver, confusion, disorientation, and seizures with cerebral infarcts. At autopsy, changes classical of active lymphomatoid granulomatosis (LYG) were found in the brain but those studied in the lungs and liver were in the healing phase. In addition, one cerebral focus in the right temporo-occipital lobe showed a lymphomatous transformation. Immunofluorescence studies done on this focus showed monoclonal IgG-lambda cytoplasmic staining in the perivascular infiltrate. Immunoperoxidase studies confirmed the monoclonal nature of this focus but showed a polyclonal pattern in the other foci. This finding is discussed along with the relationship between LYG and lymphoma.
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ranking = 4
keywords = nervous system
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20/26. lymphomatoid granulomatosis presenting as central neurogenic hyperventilation.

    A patient with lymphomatoid granulomatosis and focal transformation to lymphoma limited to the central nervous system presented with severe central neurogenic hyperventilation. The hyperventilation resolved as the underlying pathologic condition was treated with prednisone and cyclophosphamide.
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ranking = 1
keywords = nervous system
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