common variable immunodeficiency (cvid) and selective iga deficiency (sigad) are the most common primary antibody deficiencies. these two diseases may have coincidence in one family and sigad can progress to cvid which suggest common underlying genetic defects between sigad and cvid. this study was designed to find the prevalence of multiple cases in families of iranian patients with cvid or si...

We here report the case of a 2-year-old patient with primary central nervous system lymphoma B-cell origin. Due to their past medical history repeated respiratory tract infections and marked chemotherapy-associated toxicity infectious comorbidity, we suspected that also suffered from an inherited immune deficiency disorder. Despite lack classical pathognomonic symptoms for ataxia teleangiectasi...

Primary immunodeficiency diseases are rare hereditary conditions that usually occur at a young age; however, secondary immunodeficiency is acquired due to disease, drug treatment and is increasing in frequency among the population. Although periodontal diseases related to these conditions are secondary to other life threatening manifestations, they are very common and easily detectable by the p...

Primary immunodeficiency disorders are rarely diagnosed in adults but must be considered in the differential diagnosis of combined recurrent infections and autoimmune disease. We describe a patient with CTLA-4 haploinsufficiency and an abnormal regulatory T-cell phenotype. Unusually, infections were more severe than autoimmunity, illustrating therapeutic challenges in disease course.

The antigen receptor repertoires of B- and T-cells form the basis of the adaptive immune response. The repertoires should be sufficiently diverse to recognize all possible pathogens. However, careful selection is needed to prevent responses to self or harmless antigens. Limited antigen receptor repertoire diversity leads to immunodeficiency, whereas unselected or misdirected repertoires can res...