Small-bowel changes in recurrent ulceration of the mouth.

Jejunal biopsies of twenty-four patients with aphthous ulcers in whom malabsorption was not found, were studied. Four patients (16%) had subtotal villous atrophy and this structural change reversed after gluten withdrawal. There was a significant increase in intra-epithelial lymphocytes in the aphthous patients compared with healthy controls (p less than 0.001). The lymphocytic infiltration of the epithelium was higher, even in specimens with normal morphology (p less than 0.001). The findings suggest that a significant number of patients with recurrent aphthous stomatitis may have a mild form of gluten enteropathy.

Mast cells in Whipple's disease.

Abundant degranulating mast cells were observed during an ultrastructural study of jejunal biopsy from an untreated patient with Whipple's disease. Some of these cells were observed in intimate contact with macrophages containing bacteria in various stages of disintegration. Thereafter bacilli were often seen within the cytoplasm of mast cells, enclosed within vacuoles. These findings are suggestive of a cooperation between mast cell and macrophage in the defence of the organism against Whipple's bacillus.

Barium 'granuloma' of the rectum. A light and electron microscopic analysis.

A barium granuloma of the rectum, observed 6 and 9 months after a barium enema, was studied by light and electron microscopy. Mononucleated macrophages filled with electron dense material were located between the glands and the muscularis mucosae. No electron dense material was observed in the endothelial and epithelial cells. The difficulties in the identification of the material can in part be overcome with the use of electron microscopy.

Macroglobulinemia and small intestinal disease. A case report with review of the literature.

Involvement of the gastrointestinal tract in macroglobulinemia is exceedingly rare. We describe a patient with IgM biclonal immunoglobulin disorder associated with diffuse lymphoplasmacytic infiltration of the small intestine. This chronic illness was characterized by diarrhea, steatorrhea, and intestinal pseudo-obstruction. Full-thickness biopsy specimens of the jejunum showed stunted and fused villi, giardiasis, and a widespread cellular infiltrate in the lamina propria extending through the muscularis mucosae into the submucosa and muscular layers. The infiltrate had a cytologically benign appearance that was shown to be polyclonal by immunochemical stains. Intestinal vacuolated plasma cells were occasionally observed in electron microscopic study. The patient has not developed the features of Waldenstrom's disease on a clinical follow-up of 14 years.

Atypical thyroid carcinoma: atypical follicular or atypical medullary carcinoma?

The authors present a case of an atypical thyroid carcinoma with characteristics which, at light microscopy, are suggestive of a medullary carcinoma. However, ultrastructural study suggests an atypical follicular carcinoma. The differential diagnosis is discussed.

Whipple's disease. Report of a case with clinical immunological studies.

A case of Whipple's disease is described in which humoral and cellular immunity were tested. Histologic ultrastructural and immunocytochemical study were performed, before, during and after treatment. Cutaneous anergy was the main immunological dysfunction observed. During active Whipple's disease the patient had consistently negative skin tests for all antigens tested. This abnormality of delayed hypersensitivity regressed during treatment together with clinical remission of the disease. These findings are considered as secondary to Whipple's disease.

Mediastinal and hilar fibrosis.

A case is described of mediastinal and hilar fibrosis in a woman aged 22 years. The fibrotic mass compressed the lobar arteries as well as the veins of various lobes of both lungs. These large vessels as well as numerous smaller arteries and veins were to a large extent obstructed by organized thrombi. It seems likely that 3 years after the beginning of symptoms the fibrosing process was still active. The case provides some support for an immunopathological aetiology of this condition.

Ulcerative colitis, primary biliary cirrhosis, and chronic pancreatitis: coincident or coexistent?

We report a 45-year-old woman with chronic ulcerative colitis complicated by primary biliary cirrhosis and concomitant chronic pancreatitis. The combination of primary biliary cirrhosis and ulcerative colitis has only rarely been reported, and the association of these diseases with chronic pancreatitis is even rarer. These systemic manifestations of ulcerative colitis should be looked for more often.

Atypical plasma cells in alpha-chain disease.

Pathologic features of the jejunal mucosa of a patient with alpha-chain disease in its early transition to malignant stage, are described. Ultrastructural alterations were seen in some plasma cell, close similar to those described in myeloma plasma cells. Ultrastructural studies of biopsy specimens in alpha-chain disease, may permit recognition of its early transition to malignant stage and thereby encourage earlier use of cytotoxic drugs.

Hodgkin's disease with predominant infradiaphragmatic involvement and massive invasion of the bone marrow. A necropsic study of nine cases.

The authors report the results obtained in the necropsic study of nine cases of the so-called acute, visceral form of Hodgkin's disease (HD). Most of the patients (six men and three women, ranging from 42 to 74 years of age) lacked peripheral lymphadenopathies and had fever, weight loss, abnormality of hepatic function, and pancytopenia. Mixed cellularity was diagnosed in two, diffuse fibrosis in four, and reticular subtype of lymphocyte depletion in three cases. Despite the predominant infradiaphragmatic involvement, supradiaphragmatic lymph nodes were involved in six and tonsils in three cases. Spleen and bone marrow were involved in eight cases and the liver was involved in seven cases. In four cases there were also lesions in other extralymphoid organs. The involvement of the bone marrow was widespread and showed concurrent myelofibrosis and/or other signs of hematopoietic disturbance. There was a close relationship between the presence of vascular invasion (seven cases) and the extent of HD spread. It is concluded that despite its peculiarity, this form of HD fits the classic model of unicentric origin, lymphogenic contiguous spread, and hematogeneous dissemination, and should not be identified with any particular histologic type of HD.