Chronic diarrhea associated with high serum level of immunoglobulin A and diffuse infiltration of plasma cell in small intestine: A case report.

RATIONALE: Chronic diarrhea in adult patients due to various causes is very common in clinic, but patient suffering with mal-absorption due to immunoproliferative small intestinal disease was rarely reported in China. PATIENT CONCERNS AND DIAGNOSES: A 35-year-old female presented with more than three years history of chronic diarrhea, rickets, high serum value of immunoglobulin A protein, and anemia. Bone marrow aspiration suggested that the patient was in a sideropenic and megalobastic anemia stage. Duodenal and ileac biopsies revealed atrophy and blunting villi. The bowel lamina propria was infiltrated with slightly increased intraepithelial lymphocytes and mainly with diffuse plasma cells. The following enzyme labeling immunohistochemistry results were strongly positive to alpha-heavy-chain. Computed tomography manifested she had diffuse thickening of small intestine wall. At last a diagnosis of immunoproliferative small intestinal disease was made. INTERVENTIONS AND OUTCOMES: On the first month, the patient was treated with vitamin D supplements, calcium, magnesium, potassium, iron, folic acid, mecobalamin replacements and microflora probiotics. The patient frequency of water diarrhea alleviated slightly, but her weight loss, anxiety neurosis and other disorders were still severe. After taking with prednisone (40 mg per day, and gradually reduced to the lowest dose) for another month, the symptoms was gradually subsided. LESSONS: The study shows that immunohistochemical staining for alpha-heavy chain proteins should be completed on small intestine biopsy specimens if the patient is suspected a diagnosis of immunoproliferative small intestinal disease.

Cells expressing intracytoplasmic immunoglobulins in secreting and hyposecreting cases of alpha chain disease.

Alpha chain disease proteins (ACDP) originated probably from secreting plasma-cells predominantly present in diffuse and massive enteromesenteric lymphoid infiltration. Very decreased levels of abnormal alpha chain molecules were detected in sera of patients with immunoblastic lymphoma occurring in the late course of the disease. A direct correlation might exist between the proportion of cells bearing intracytoplasmic IgA determinants and the serum amounts of alpha chain disease protein. Relevant evidence raised from study of proliferating lymphoid cells using the unlabeled peroxidase anti-peroxidase method of immunocytochemistry. The percentage of cells expressing intracytoplasmic alpha chains was found to be greater readily secreting case than in hyposecreting case of alpha chain disease. Furthermore, the cells from secreting situation exhibited much more pronounced specific staining, indicative of probably more active synthesis state. Taken together with histological data, these results suggested a possible late evolutionary pathway without detectable intracytoplasmic and serum alpha chain disease protein. They might also support the hypothesis that alpha chain disease and mediterranean lymphoma were different evolutionary phases of the same entity.

Massive plasma cell infiltration of the digestive tract. Secretory component as the rate-limiting factor of immunoglobulin secretion in external fluids.

A 29-yr-old Tunisian man had a clinical immunoproliferative small intestinal disease, different from alpha-chain disease. Serum contained 52.5 mg/ml of polymeric immunoglobulin A (IgA). Immunohistochemistry revealed a massive diffuse polyclonal IgA (99%)-plasma cell infiltration in the small bowel mucosa, with a smaller increase of IgA-producing cells in gastric and colonic mucosae. Secretory IgA levels were normal in jejunal and bronchoalveolar secretions. However, both fluids contained polymeric IgA devoid of secretory component, and free secretory component was absent. This suggests that secretory component was the limiting factor in transport of IgA in the secretions. A relative deficiency in secretory component, as compared with the huge supply of polymeric IgA, may have limited the secretory component-mediated active transport of IgA into secretions. This resulted in the appearance of high levels of polymeric IgA, unlinked to secretory component, both in serum and in the jejunal and bronchoalveolar fluids.

Immunoproliferative small-intestinal disease: clinical features and outcome in 30 cases.

Experience with 30 patients with immunoproliferative small intestinal disease followed prospectively between 1971 and 1986 is described. All presented with malabsorption or growth retardation and had similar clinical, biochemical, and radiological features, irrespective of the presence of lymphoma or immunological abnormality. Alpha-chain disease protein was detected in 4 of the 11 patients who had a non-lymphomatous, predominantly plasmacytic infiltration of the small bowel; and in 5 of the 19 cases with diffuse intestinal lymphoma. The importance of exploratory laparotomy to include full-thickness intestinal biopsy in patients who have a benign infiltrate on peroral biopsy is demonstrated by the finding of lymphoma in operative specimens in 9 of 15 patients with mature, lymphoplasmacytic cells, and 5 of 8 patients with atypical, lymphoplasmacytic cells. The majority of patients with fully established benign disease, even those elaborating alpha-chain disease protein, appeared to have a good prognosis. No patient with immunoproliferative small intestinal disease developed immunologically demonstrated alpha-chain disease or frank lymphoma, when this was not found initially at explorative laparotomy.

[Mediterranean type of primary lymphoma in the small intestine - a diagnostic problem]. / Mediterraner Typ des primären Dünndarmlymphoms--Ein diagnostisches Problem.

In primary lymphoma of the gastro-intestinal tract radiological and clinical findings are often uncharacteristic for a long time and even tissue biopsy may be difficult to classify pathohistologically. The conclusive diagnosis of this rare entity can be established radiologically only in combination with serological, endoscopic und pathologic findings during the course of the disease.