Immunoglobulin deficiencies

S. pneumoniae H influenzae taxime or ceftriaxone Sickle cell disease Immunoglobulin deficiency... 

Type I Immunoglobulin Deficiency in Burkitt s Lymphoma Low IgM WITH Normal IgG (mg/ml) ... 

In twelve of sixty-three patients, both the serum IgM and the serum IgG concentrations were depressed. Two of these Burkitt s lymphoma children also had low IgA, but in the other 10 children the scrum IgA was normal. There were 5 known deaths in children classified with this type II deficiency. Seven of these children had tumor classification of the grade III type III immunoglobulin deficiency. There were 14 children with Burkitt s lymphoma classified with this type III immunoglobulin deficiency with moderately reduced levels of scrum IgG and normal levels of IgM. One child in the grouji had a moderately elevated IgA,... 

Adverse effects include local tenderness, muscle soreness or stiffness at the injection site, low grade fever, sensitisation to repeated injections of human globulin in immunoglobulin deficient patients. 

Given prophylactically or soon after exposure to hepatitis A given to prevent or modify measles (Rubeola), Varicella (Zoster), Rubella, and immunoglobulin deficiency... 

IGIV in high doses (2 g/kg) has proved effective in a variety of different conditions ranging from immunoglobulin deficiencies to... 

In a nested case-control study in Mexican patients with rheumatoid arthritis encompassing 1274 patient years, the risk factors were determined for acquiring infectious diseases (378). In addition to the cumulative doses of methotrexate and the duration of corticosteroids use, the mean daily dose of penicillamine was a risk factor. In one patient the infection was secondary to neutropenia. Tests for a possible immunoglobulin deficiency were not performed. 

Immunoglobulin deficiency Multiple myeloma Nephrotic syndrome Systemic lupus erythematosis... 

In this group the immunoglobulin deficiency is thought to result from circulating factors suppressing immunoglobulin synthesis. Renal failure (H19, H25) celiac disease (AlO, B16, H38), diabetes mellitus (Bll, H25), severe infection (H25), and rubella in utero (H25, P9, S22) can have this result. Immunosuppressive (cytotoxic) treatment produced severe AG in only 1 of 54 patients, although DG resulted in another 4. Thus while such treatment is well known to predispose to severe infection... 

The same sequence of suppressed synthesis, IgM, then IgA, then IgG to result in pattern 9 Fig. 10 is characteristic of the commonest predisposition to immunoglobulin deficiency, lymphoid neoplasia. The sequence may take years as shown in Fig. 8, where the highest incidence and most severe DG and/or AG is found with the neoplasias compatible... 

Fig. 8. Immunoglobulin deficiency in 206 patients with malignant reticuloses. The white boxes indicate 100% mean adult serum levels the black areas, the mean levels found in patients with malignant reticuloses. In general, deficiency becomes more severe with longer duration of disease, and affects IgM more than IgA, more than IgG. Reproduced by courtesy of the Proceedings of the Royal Society of Medicine (H25).

How does this suppression of normal immunoglobulin synthesis occur Simple displacement of the normal plasma cells by neoplastic cells seems unlikely, because in the marrow this would also result in leukoerythro-blastic anemia this in fact is found in fewer than 5% of these patients. Marrow occupation would also depress IgG first (Section 4.3), whereas this is usually the last to fall. Furthermore, IgA is largely derived from the gut, which is rarely directly involved by tumor. It has also been suggested that the neoplastic cells misappropriate available amino acids at the expense of normal plasma cells. Immunoglobulin deficiency is, however, unusual with most other types of neoplasia, and even among the... 

In a minority of patients there is a clear picture of long-standing antibody deficiency preceding the development of lymphoid neoplasia. It seems that the humoral defect results in overstimulation of the cellular mechanisms of immunity to a degree increasing the risk of mutation. Evidence that immunoglobulin deficiency can itself sometimes be primary to lymphoid neoplasia has been reviewed elsewhere (F12). 

Secondary deficiencies are 10-100 times commoner than primary deficiencies (Section 4). Among the primary immunoglobulin deficiencies, it is also clear that DG is some 6-12 times commoner than AG (H25). In general the primary deficiencies are more severe than the secondary (see Table 3). 

Since tuberculosis, bronchiectasis, emphysema, fibrocystic disease, intrinsic asthma, and hilar sarcoidosis are often associated with a dominant elevation of IgA (see Fig. 10 2), this has no specific value. Pneumocystis pneumonia in infants can elevate IgM (K7) (Fig. 10 6). Of course recurrent respiratory infection is a common mode of presentation of frank immune deficiencies (H32), and we find these in some 4% of patients with such histories. Reaginic states have already been considered under IgE globulin, and immunoglobulin deficiencies among atopic children support the hypothesis that atopic subjects may have a poor immunological dictionary (K2). Direct IgE assay of fluid from nasal polyps can also point to an allergic origin (D6). Parotid saliva normally contains only detectable IgA (see Table 1) (mixed saliva... 

B27. Buckley, R. H., and Sidbury, J. B., Hereditary alterations in the immvme response Coexistence of agammaglobulinemia, acquired hypogammaglobulinemia and selective immunoglobulin deficiency in a sibship. Pediat. Res. 2, 72-84 (1968). 

F14. Fudenberg, H. H., Kamin, R., Salmon, S., and Tormey, D. C., Quantitative abnormalities in lymphocyte metabolism in patients with immunoglobulin deficiency. In The Gamma Globulins, Nobel Symp. 3 (J. Killander, ed.), pp. 585-596. Almqvist Wiksell, Stockholm, 1968. 

W6. West, C. D., Hong, R., and Holland, N. H., Immunoglobulin levels from the newborn period to adulthood and in immunoglobulin deficiency states. J. Clin. Invest. 41, 2054-2064 (1962). 

A middle-aged man presents with a markedly enlarged tonsil and recurrent infections with serum immunoglobulin deficiency. Chromosome analysis demonstrates a translocation between the immunuglobulin heavy chain locus on chromosome 14 and an unidentified gene on chromosome 8. Which of the following is the most likely cause of his phenotype ... 

Thus, although the basic biochemical mechanisms by which a particle activates serum opsonins are not yet established, it is known that complement deficiency is frequently associated with increased susceptibility to infections (A3). Measurement of serum activation is one way of detecting phagocytic dysfunction as a consequence of opsonic disorders (SIO), such as C3 or immunoglobulin deficiency. 

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