The term inflammatory pseudotumour encompasses a very diverse group of conditions, with different sites, whose only common feature is the absence of a specific histological diagnosis. The histopathological and aetiopathogenic data have been derived from experience in the thoracic, orbital and abdominal forms, which are much more frequent than disease affecting the nasal cavities and sinuses. However, there is no evidence that these data are applicable to sino-nasal pseudotumour. We present a case of inflammatory pseudotumour of the nasal cavity and a review of the literature (19 cases in 30 years) with respect to the clinical and radiological findings, the natural history and the specific management of disease at these sites. Surgery seems to be the best option for this localization. Corticosteroids and radiotherapy may be of value in cases of residual tumour after the histological confirmation of the diagnosis and the exclusion of neoplasia.
Primary intestinal lymphangiectasia is a rare congenital condition associated with protein-losing enteropathy. Hypogammaglobulinemia and lymphopenia secondary to this condition are frequent but infectious complications are not. So far few immunological studies have been made in these patients. We report here the results of such a study carried out in two adolescents. Both patients presented with a dramatic decrease in serum gammaglobulins, especially IgG and IgA, and in peripheral blood lymphocytes, especially CD4 T helper cells. From a functional standpoint, the proliferative response to certain mitogens was reduced. A decrease in in vitro production of immunoglobulins by B lymphocytes may be due to a faulty T/B cell cooperation. Histological examination of duodenal biopsy specimens revealed a decreased number of intraepithelial lymphocytes. Colonoscopy revealed nodular lymphoid hyperplasia in the terminal ileum, confirmed by endoscopic biopsy. The role of these abnormalities in the development of infectious complications and lymphoma is underscored.
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