Pulmonary eosinophilic gramuloma in a child.
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The occurrence of pulmonary eosinophilic granuloma in a 3-year-old child is described. She presented with a pneumothorax and typical radiological changes and the diagnosis was confirmed by lung biopsy. There was no objective evidence of improvement after radiotherapy when lung function was assessed by gamma scans. She died suddenly while abroad. (+info)
Anaplastic gangliocytoma with eosinophilic cytoplasmic granules in a cow.
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In a 3-year-old Holstein cow, a tumor mass replaced the left olfactory bulb. The tumor was highly or moderately cellular, and consisted of tumor cells showing pleomorphism and anaplasia, sometimes with intracytoplasmic granules. The tumor showed weak reactivity for neurofilaments (NF) in most cells with distinct staining in a minority, and it was extremely rare to see neoplastic cells with positivity for glial fibrillary acidic protein (GFAP). The neoplastic cells displayed some ultrastructural features reminiscent of ganglionic cells, and the cytoplasmic granularity was due to the presence of numerous lysosomes. This tumor expressing both NF and GFAP may be histogenetically related to brain tumors of pluripotential cell origin in calves. (+info)
Schistosome infection of transgenic mice defines distinct and contrasting pathogenic roles for IL-4 and IL-13: IL-13 is a profibrotic agent.
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Experimental Schistosoma mansoni infections of mice lead to a dynamic type 2 cytokine-mediated pathological process. We have used IL-4-deficient, IL-13-deficient, and IL-4/13-deficient mice to dissect the role of these cytokines in the development of immune response and pathology following S. mansoni infection. We demonstrate that while both of these cytokines are necessary to develop a robust Th2 cell-driven, eosinophil-rich granuloma response, they also perform disparate functions that identify novel sites for therapeutic intervention. IL-13-deficient mice demonstrated significantly enhanced survival following infection, which correlated with reduced hepatic fibrosis. In contrast, increased mortality was manifest in IL-4-deficient and IL-4/13-deficient mice, and this correlated with hepatocyte damage and intestinal pathology. Therefore, we demonstrate that during a dynamic type 2 cytokine disease process IL-13 is detrimental to survival following infection, whereas IL-4 is beneficial. (+info)
Magnetic resonance imaging of calvarial eosinophilic granuloma with pericranial soft tissue reaction--case report.
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A 4-year-old girl presented with an eosinophilic granuloma in the cranial vault. Magnetic resonance (MR) imaging showed the mass as slightly low intensity on T1- and high intensity on T2-weighted images. The pericranial soft tissue was densely enhanced after gadolinium-diethylenetriaminepenta-acetic acid infusion. The mass was soft and successfully removed. Histological examination disclosed Langerhans' cell histiocytosis. MR imaging is useful for the diagnosis of calvarial eosinophilic granuloma with soft tissue involvement. (+info)
Differentiation of Langerhans cells in Langerhans cell histiocytosis.
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Langerhans cell histiocytosis (LCH) consists of lesions composed of cells with a dendritic Langerhans cell (LC) phenotype. The clinical course of LCH ranges from spontaneous resolution to a chronic and sometimes lethal disease. We studied 25 patients with various clinical forms of the disease. In bone and chronic lesions, LCH cells had immature phenotype and function. They coexpressed LC antigens CD1a and Langerin together with monocyte antigens CD68 and CD14. Class II antigens were intracellular and LCH cells almost never expressed CD83 or CD86 or dendritic cell (DC)-Lamp, despite their CD40 expression. Consistently, LCH cells sorted from bone lesions (eosinophilic granuloma) poorly stimulated allogeneic T-cell proliferation in vitro. Strikingly, however, in vitro treatment with CD40L induced the expression of membrane class II and CD86 and strongly increased LCH cell allostimulatory activity to a level similar to that of mature DCs. Numerous interleukin-10-positive (IL-10(+)), Langerin(-), and CD68(+) macrophages were found within bone and lymph node lesions. In patients with self-healing and/or isolated cutaneous disease, LCH cells had a more mature phenotype. LCH cells were frequently CD14(-) and CD86(+), and macrophages were rare or absent, as were IL-10-expressing cells. We conclude that LCH cells in the bone and/or chronic forms of the disease accumulate within the tissues in an immature state and that most probably result from extrinsic signals and may be induced to differentiate toward mature DCs after CD40 triggering. Drugs that enhance the in vivo maturation of these immature DCs, or that induce their death, may be of therapeutic benefit. (+info)
Eosinophilic granuloma. A different behaviour in children than in adults.
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Localised Langerhans-cell histiocytosis of bone (eosinophilic granuloma) is a benign tumour-like condition with a variable clinical course. Different forms of treatment have been reported to give satisfactory results. However, previous series all contain patients with a wide age range. Our aim was to investigate the effect of skeletal maturity on the rate of recurrence of isolated eosinophilic granuloma of bone excluding those arising in the spine. We followed up 32 patients with an isolated eosinophilic granuloma for a mean of five years; 17 were skeletally immature. No recurrences were noted in the skeletally immature group even after biopsy alone. By contrast, four of 13 skeletally mature patients had a recurrence and required further surgery. This suggests that eosinophilic granuloma has a low rate of recurrence in skeletally immature patients. (+info)
Cranial fasciitis of childhood.
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Cranial fasciitis is a benign fibroblastic tumor of the skull found almost exclusively in young children. It is histologically identical to nodular fasciitis. We present the clinical, radiologic, and pathologic findings of a 7-month-old male infant with cranial fasciitis of the skull. Herein, we include the first description of this entity's diffusion-weighted imaging appearance. Although rare, cranial fasciitis can mimic more aggressive processes both clinically and radiographically. (+info)
GASTRO-DUODENAL CROHN'S DISEASE.
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Gastro-duodenal Crohn's disease is rare. Thirty-one previously reported cases are briefly reviewed; histological confirmation of the diagnosis was not always possible. Details are given of a patient with pyloro-duodenal involvement accompanied by terminal ileitis and appendicitis where surgical specimens were available for study. The differential diagnosis is considered from the clinical and pathological aspects. (+info)