Immune Deficiency States Research Articles

The pathology of so-called Mediterranean abdominal lymphoma with malabsorption.

Surgical specimens, postmortem material, or both from 22 patients with the syndrome designated as “Mediterranean abdominal lymphoma with malabsorption” were studied in detail. All patients exhibited a malabsorption syndrome. In most of those carefully followed from the onset of the disease, the syndrome seemed to have preceded the development of palpable abdominal masses. In 20 of the 22 patients, intestinal tissues were available for histologic study. In all specimens, diffuse severe plasma cell infiltrations were evident in the intestinal mucosa and submucosa. Malignant lymphomas in the form of single or multiple circumscribed intestinal tumors occurred in 14 of these 20 patients and malignant lymphomas of the mesenteric lymph nodes in two. In four patients, no malignant lymphomas were evident. These observations suggest that the diffuse plasma cell infiltrations, rather than the malignant lymphomas, were responsible for the malabsorption syndrome. There was no morphological evidence that the malignant lymphomas observed in 75% (16 of 20) of the patients were histogenetically related to the diffuse plasma cell infiltration. The possibility is suggested that this abnormal, though not provably neoplastic, proliferation of plasma cells is a morphological manifestation of an immune deficiency state which predisposes the patients to the development of malignant lymphoreticular neoplasms.

Immune Deficiency States Associated with Malignant Disease in Man

Immune Deficiency States Associated with Malignant Disease in Man

Listeriosis in humans: an evaluation.

Infection of man with Listeria monocytogenes was first described in 1929 [1], and in recent years it has been reported with increasing frequency.1 L. monocytogenes has been isolated and identified in many species of animals [2] and it was first thought that non-human hosts were the principal source for human infections. Most recent cases, however, in this country have been in urban residents with no known animal contacts [3]. Later, because many of the isolations from humans were from neonates and the products of conception, it was thought to be primarily an obstetric and pediatric disease [4]. Still later, Louria et al. [5] called attention to the association between infection with Listeria and lymphoproliferative diseases. Subsequent reports by Simpson et al. [6] and Buchner and Schneierson [3] supported this association. Because of these reports, the concept has become prevalent that listerial infection occurs almost exclusively as a complication of an underlying primary disorder (malignancy, cirrhosis, immune deficiency states). This communication endeavors to put listeriosis into a more realistic perspective. That is, although many infections occur in infants and in patients with an underlying disease, most occur in previously healthy people of all age groups and are unrelated to a rural setting or animal exposure.

Incorporation of L-leucine-14C into immunoglobulins by jejunal biopsies of patients with celiac sprue and other gastrointestinal diseases.

Incorporation of L-leucine-(14)C into proteins and immunoglobulins in vitro was determined in jejunal biopsy specimens from normal volunteers, patients with celiac sprue before and after introduction of gluten into the diet, patients with Whipple's disease in remission, and patients with immune deficiency states. Values for incorporation of L-leucine-(14)C into total and soluble protein by biopsies from five celiac sprue patients on a gluten-free diet were within, or slightly above, the 95% confidence limits for control data. One patient with celiac sprue and with normal intestinal histology had a normal value for incorporation into IgA; the other four patients with flat mucosas had elevated values. In Whipple's disease in remission, values for incorporation into total protein and IgA were within the control limits, whereas incorporation into soluble protein was increased. Patients with hypogammaglobulinemia or IgA deficiency had normal or elevated values for incorporation into total and soluble proteins; in these cases, however, no incorporation into IgA was detected. Biopsies from the four celiac sprue patients studied revealed that with introduction of gluten into the diet (a) incorporation into total protein, soluble protein, or both, increased; (b) incorporation into IgA increased in all patients, and in two instances the increase was greater than the increase in incorporation into total protein; and (c) incorporation into IgM increased in all patients. The changes during gluten administration usually occurred before changes in gastrointestinal absorptive function or in concentration of IgA in serum could be detected. These results indicate that gluten challenge stimulates increased local intestinal synthesis of immunoglobulins in patients with celiac sprue. The reaction occurs within days and it is possible that it plays a primary role in the pathogenesis of the disease.

Immune deficiency state in a girl with eczema and low serum IgM. Possible female variant of Wiskott-Aldrich syndrome.

This report concerns an immune deficiency disorder in a girl with eczema. She has had recurrent infections including three severe attacks of herpes simplex and five attacks of pneumococcal meningitis. There is a moderate lymphopenia, dysgammaglobulinaemia with high IgG, high IgA, and low IgM; lymphocyte transformation with phytohaemagglutinin is impaired. Production of circulating antibody is abnormal, as are delayed hypersensitivity reactions. Although there is no thrombocytopenia, the resemblance to the Wiskott-Aldrich syndrome is discussed.

Experimental background to immune deficiency states.

Experimental background to immune deficiency states.

Immunological deficiency and malignant lymphoma.

Immunologkal studies suggest that Hodgkin's disease is the result of proliferation of a thymus dependent population of lymphocytes and that lymphatic leukemia is the result of a proliferation of a non-thymus-dependent population of lymphocytes. Certain congenital immune deficiency states are associated with a high incidence of malignant lymphoproliferative diseases. Such congenital diseases are overtly present in few patients with malignant lymphomas; however, evidence is adduced to show that there are physiological periods of relative immune deficiency and that these periods differ for the 2 populations of lymphocytes. The author hypothesizes that susceptible individuals are at increased risk of developing malignant lymphomas during these periods. This hypothesis helps to explain the wide difference in peak age distribution between lymphatic leukemia (the very young with acute lymphoblastic leukemia and the very old with chronic lymphocytic leukemia) and Hodgkin's disease (the adolescent and young adult).

Repeated infections with respiratory syncytial virus: Their occurrence, and the significance of antigenic variations of virus in such reinfections

Tomasi and others have shown that the major immune globulin constituent of various body secretions, including saliva, bronchial, and nasal secretions, is IgA. Studies in our laboratory have indicated that there is a selective mechanism for IgA, which involves the addition of an antigenically distinct protein, the transport to the IgA molecule. Patients with absence of IgA, the agammaglobulinemics treated with IgG, and ataxia-telangiectasia patients, are susceptible to infections of the respiratory tract. Although they have no IgA they do have the piece free in the saliva. Children, who have not yet developed normal adult levels of IgA in serum or saliva, also have free piece in saliva. Three teen-aged patients (two of whom are siblings) with unexplained chronic otitis media, sinusitis, bronchitis, and bronchiectasis, were studied. They had low salivary IgA relative to the adult normal of 1 to 3 mg. per cent. Age-specific normal values have not been established. These findings indicate that there is a local antibody defense system which involves the IgA class of antibodies. Defects are possible at 3 points in the mechanism: (1) production of IgA in plasma cells either locally or in distant parts of the body, (2) production of the piece, (3) their coupling together and subsequent secretion into the salivary collecting ductules. A defect at any of these points would result in a local immune deficiency state.