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MeSH Review:

Dysgammaglobulinemia

Kühlmann-Rabens, I. et al., Sun, T. et al., Verbrugh, H.A. et al., Alangari, A. et al., Braig, D.U. et al., et al.

Alangari, Abobaker, Kanegane, Miyawaki,

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Disease relevance of Dysgammaglobulinemia

X-linked lymphoproliferative disease is a rare immunodeficiency disorder characterized by extreme vulnerability to Epstein-Barr virus, dysgammaglobulinemia, and very high incidence of lymphoma [1].
Alpha-1-antitrypsin (AAT) levels were measured in patients with three types of dysgammaglobulinemia: monoclonal gammopathy, polyclonal hypergammaglobulinemia and hypogammaglobulinemia [2].

High impact information on Dysgammaglobulinemia

We report the results of a genome-wide scan in three multiplex families with CVID, IgAD, and dysgammaglobulinemia, where affection is inherited in an autosomal dominant pattern [3].
Vitiligo and dysgammaglobulinemia. A case report and family study [4].
Beside the appearance of high levels of IgG in dysgammaglobulinemic chickens during the first weeks of life and in transitory dysgammaglobulinemia, remarkable IgG synthesis can be temporarily induced by the mitogenic activity of LPS and even more by the regulatory function of Levamisole [5].
Acquired alpha-1-antitrypsin deficiency and dysgammaglobulinemia [2].
Except for 1 patient with dysgammaglobulinemia, sera from the patients contained normal amounts of immunoglobulins and complement (CH50 and C3), and they all effectively opsonized Staph. aureus [6].

Biological context of Dysgammaglobulinemia

Mice lacking both IL-4 and IL-21R exhibited a significantly more pronounced phenotype, with dysgammaglobulinemia, characterized primarily by a severely impaired IgG response [7].

References

X-linked lymphoproliferative disease associated with hypogammaglobulinemia and growth-hormone deficiency. Alangari, A., Abobaker, A., Kanegane, H., Miyawaki, T. Eur. J. Pediatr. (2006) [Pubmed]
Acquired alpha-1-antitrypsin deficiency and dysgammaglobulinemia. Sun, T., Evans, H.E., Degnan, T. Ann. Clin. Lab. Sci. (1980) [Pubmed]
Linkage of autosomal dominant common variable immunodeficiency to chromosome 5p and evidence for locus heterogeneity. Braig, D.U., Schäffer, A.A., Glocker, E., Salzer, U., Warnatz, K., Peter, H.H., Grimbacher, B. Hum. Genet. (2003) [Pubmed]
Vitiligo and dysgammaglobulinemia. A case report and family study. Bader, P.I., Biegel, A., Epinette, W.W., Nance, W.E. Clin. Genet. (1975) [Pubmed]
Attempts to localize the defect in dysgammaglobulinemia of UM-B19 chickens by studying the effect of immunomodulating substances on immunoglobulin and antibody production. Kühlmann-Rabens, I., Wanke, R., Storandt, F., Altmann, B., Lösch, U., Merkenschlager, M. Vet. Immunol. Immunopathol. (1987) [Pubmed]
Phagocytic and chemotactic function of polymorphonuclear and mononuclear leucocytes in patients with recurrent staphylococcal infections. Verbrugh, H.A., van Dijk, W.C., Hendrickx, G.F., van der Stadt, D., Stoop, J.W., Peterson, P.K., Verhoef, J. Scand. J. Infect. Dis. (1980) [Pubmed]
A critical role for IL-21 in regulating immunoglobulin production. Ozaki, K., Spolski, R., Feng, C.G., Qi, C.F., Cheng, J., Sher, A., Morse, H.C., Liu, C., Schwartzberg, P.L., Leonard, W.J. Science (2002) [Pubmed]

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