Low Serum Immunoglobulin Research Articles

Angioimmunoblastic lymphadenopathy with hypogammaglobulinemia: Possible role of monocyte suppression

A patient with angioimmunoblastic lymphadenopathy had low serum immunoglobulin values and no antibodies to injected immunogens. This occurred despite the proliferation of polyclonal B cells. T cells were deficient in number and in lymphoproliferative responses, but their helper and suppressor functions were maintained, la-antigen bearing leukocytes from the patient stimulated poorly in mixed leukocyte culture. In vitro immunoglobulin synthesis by mononuclear leukocytes from the patient was severely impaired. These leukocytes actively suppressed immunoglobulin synthesis by normal cells from healthy subjects in co-culture. The responsible cell had characteristics of a monocyte. The suppression was selective for humoral immunity and was manifest despite normal numbers of monocytes. It appears that heterogeneous immunoregulatory abnormalities can underlie the syndrome of angioimmunoblastic lymphadenopathy. Furthermore, monocyte suppressor abnormalities may be implicated in clinical disease phenomena.

Colostrum and Serum Immunoglobulin Levels in Jersey Cattle

The present results confirm that Jersey cows have low serum immunoglobulin levels compared to other breeds. However, their colostrum levels are similar and when Jersey calves receive their dams’ colostrum they can acquire comparable serum levels of immunoglobulin to calves of other breeds. It would appear therefore that the high mortality rate in Jersey calves compared with other breeds cannot be accounted for solely on the basis of their serum immunoglobulin levels although the practice of a limited drying-off period prepartum could be significant in some instances.

Pulmonary alveolar proteinosis in two siblings with decreased immunoglobulin A

A brother and sister with classic, biopsy proved pulmonary alveolar proteinosis are described. Both had low serum and low normal secretory immunoglobulin A (IgA) levels. A tendency for familial occurrence is possible and it is recommended that patients with pulmonary alveolar proteinosis, and their families, be evaluated for immunologic deficiencies.

Parainfluenza pneumonia in severe combined immunodeficiency disease

IN PATIENTS with severe combined immune deficiency, death is often secondary to pneumonitis. Most reports refer to Pneumocystis carinii or bacteria as the common pathogen, whereas viral infections are rarely described as playing a major role in these patients. This paper describes two infants with SCID who developed parainfluenza virus pneumonitis, ending in respiratory failure and death. CASE REPORTS Patient 1. This 9-week-old white girl was transferred to the Hospital for Sick Children for evaluation following two episodes of possible neonatal sepsis. Fever, poor feeding, and lethargy were observed during the first days of life. All cultures for bacteria were negative and, after completing a course of antibiotics, she was discharged. At five weeks of age she was admitted for treatment of possible osteomyelitis. Laboratory investigations revealed abnormally low serum immunoglobulin levels and impaired T-cell function, and she was transferred to HSC for evaluation. On admission she was found to be less than the third percentile for both length and weight. Physical examination revealed an absence of lymphoid tissue, hypertonia, and multiple inflamed iv sites on the head and extremities. Immunologic evaluation confirmed a combined humoral and cellular immune deficiency with profound hypogammaglobulinemia, less than 10% E-rosetring T-lymphocytes, and absent proliferative responses to phytohemagglutinin, concanavalin A, pokeweed mit0gen, and allogeneic cells. Parenteral gamma globulin replacement was begun. On admission a chest radiograph revealed bilateral upper lobe consolidation. The initial nasopharyngeal aspirate revealed

Lymphocyte subpopulations in X-linked severe combined immunodeficiency (SCID): Evidence against a stem cell defect. Transformation response to calcium lonophore A23187

Lymphocytes from a patient with severe combined immunodeficiency (SCID) of the X-linked adenosine deaminase-positive type were studied in detail. Eighty per cent of peripheral blood cells were positive for surface immunoglobulins, the predominant fluorescence being immunoglobulin G (IgG). Double-labeling experiments showed 30 per cent of cells to be positive for γ and μ; all μ-staining cells also had γ. Few δ-positive cells were found. Both the γ- and μ-bearing cells contained either κ and λ light chains, the ratio of κ to λ being 4:1. These high percentages of immunoglobulin-bearing cells are in contrast to the very low or absent serum immunoglobulin concentrations and suggest failure of differentiation into actively-secreting lymphocytes or plasma cells. No evidence for circulating suppressor cells was found. An unusual subpopulation of B cells, previously termed B 2, predominated in that complement receptors C3b and C3d were absent by EAC and Raji technics. Thymus-derived lymphocytes were absent in this patient, and T-cell functions were severely impaired as measured by skin test anergy, and mitogen and allogeneic responses in vitro. An increased tridiated thymidine ( 3H-TdR) incorporation and a normal percentage of cells entering interploid S phase and tetraploid G 2 and M phase of the cell cycle (S-G2-M) (by cytofluorographic analysis) followed the incubation of lymphocytes with calcium ionophore A23187. These membrane findings suggest possible pathogenetic mechanisms for the deficiencies in patients with this specific subset of SCID disease. The absence of a putative endogenous membrane ionophore, a defect in cytoskeleton, for example, a defect in microtubules or microfilaments, or impaired intracellular cyclic nucleotide activation or distribution, or defects in protein phosphorylation or sialation could all explain these observations.

Idiopathic Late-Onset Immunoglobulin Deficiency With Functional T-Cell Deficiency

The common features in our two patients were late onset of infections that are known to complicate both T-cell and B-cell deficiencies, decreased numbers of circulating B-cells with low serum immunoglobulin levels, and normal numbers of circulating T-cells, which were, however, defective in the response to delayed hypersensitivity skin test antigens and to mitogens in vitro. The composite of clinical and immunologic aberrations is consistent with the presence of an immune deficiency involving the B-cell system quantitatively and the T-cell system qualitatively.

Lymphocytes in human immunodeficiency states: a study of membrane‐associated immunoglobulins

AbstractThe peripheral blood lymphocytes from fourteen patients with various types of immunodeficiency states were studied with respect to surface immunoglobulins by the fluorescent antibody technique. The results obtained ranged from a complete absence of immunoglobulin‐bearing lymphocytes in certain patients to normal levels in others. Serial determinations in each patient gave similar results. Three male children with the X‐linked form of the disease and very low serum immunoglobulins had no lymphocytes that stained for immunoglobulins. However, two brothers and a third boy who presumably also had this type of disease, had immunoglobulin‐bearing lymphocytes in the low normal range. Of special interest was an adult female patient with a thymoma who showed no such lymphocytes. Patients with the common variable type of immunodeficiency all had some staining lymphocytes numbering from a very few to within the normal range.The findings indicate that this approach provides important information regarding the basic defects in certain of these patients, and various hypotheses are proposed.

Decreased Rate of Synthesis of Immunoglobulin (IgG) in Rats Infected with Moloney Leukemia Virus

Summary Rats infected with Moloney virus when newborn and uninfected control animals were injected with IgG prepared from normal inbred Osborn-Mendel rat serum and labeled with 131I. The decline in whole body and blood radioactivity of infected and control animals was measured for a period of 2 weeks and the biologic half-life of the labeled IgG was determined. From these and other data, the synthetic rates for each group of animals was calculated. Infected animals, which had reduced serum IgG levels, had a reduced synthetic ability rather than an increased catabolism of IgG molecules, and this could account for their low serum immunoglobulin levels. The defect in synthetic ability was most pronounced in hyperimmunized animals, resulting in a calculated synthetic rate of 7.5 mg IgG/day in infected rats and 53 mg/day in control rats.

The congenital agammaglobulinemias. A heterogeneous group of immune defects.

THE FIRST type of agammaglobulinemia reported was an X-linked recessive disease characterized by recurrent bacterial infections, very low serum immunoglobulins,* and absence of antibody responses. 1 Many other types have since been recognized 2-5 and a short classification of the agammaglobulinemias is listed in Table 1. Each type appears to be distinct, and each is probably the result of a different defect in the maturation and function of immunologic mechanisms. The purpose of this report is to present studies of seven patients with agammaglobulinemia. These studies indicate that cases currently called congential sporadic agammaglobulinemia may be a heterogeneous group with different defects of immunity. Agammaglobulinemia is used here in the generic sense to mean a group of related diseases which are characterized by low serum immunoglobulins, impaired antibody response, and abnormal lymphoid morphology. The use of agammaglobulinemia in this sense is comparable to the use of anemia to indicate diseases