Adult Primary Immune Deficiency: What Are We Missing?

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Presentation transcript:

Adult Primary Immune Deficiency: What Are We Missing? Bharat T. Srinivasa, Reza Alizadehfar, Martin Desrosiers, Joseph Shuster, Nitika Pant Pai, Christos M. Tsoukas  The American Journal of Medicine  Volume 125, Issue 8, Pages 779-786 (August 2012) DOI: 10.1016/j.amjmed.2012.02.015 Copyright © 2012 Terms and Conditions

Figure 1 Diagnostic algorithm and classification by presence and type of immune deficiency. ESID/PAGID=European Society for Immunodeficiencies and the Pan-American Group for Immunodeficiency. The American Journal of Medicine 2012 125, 779-786DOI: (10.1016/j.amjmed.2012.02.015) Copyright © 2012 Terms and Conditions

Figure 2 Comparison of the serum IgG of each group of patients with humoral immune deficiencies, including those of the 2 novel groups. All serum samples were obtained before any immunoglobulin replacement therapy for patients with common variable immune deficiency. IgG levels of individuals with IgA deficiency were in the normal range (7-16 g/L) and similar to those of the nonimmune-deficient individuals. Median levels of IgG in the B-cell lymphopenia group were borderline normal and significantly higher than those with hypogammaglobulinemia. Significant differences were noted between the IgG levels of the nonimmune-deficient individuals and those with B-cell lymphopenia, as well as those with hypogammaglobulinemia. Ig=immunoglobulin. The American Journal of Medicine 2012 125, 779-786DOI: (10.1016/j.amjmed.2012.02.015) Copyright © 2012 Terms and Conditions

Figure 3 Comparison of age distributions of groups of patients with humoral deficiencies with those without immune deficiency. B-cell lymphocytopenic patients were significantly older than individuals in all groups at their first clinic visit, with a median age of 67 years. The onset of symptoms that were attributed to the immune deficiency occurred later in life. In contrast, the common variable immune-deficient patients had the lowest median age at their first clinic visit, and the clinical onset of immune deficiency occurred in young adulthood. ns=not significant. The American Journal of Medicine 2012 125, 779-786DOI: (10.1016/j.amjmed.2012.02.015) Copyright © 2012 Terms and Conditions

Figure 4 Comparison of relative and absolute numbers of natural killer cells in patients with natural killer lymphocytopenia and in nonimmune-deficient patients. There was a highly significant difference between the groups in the percentage of circulating natural killer cells and in the absolute values. The American Journal of Medicine 2012 125, 779-786DOI: (10.1016/j.amjmed.2012.02.015) Copyright © 2012 Terms and Conditions