Hereditary deficiencies of early complement components have usually been associated with the development of rheumatic diseases like systemic lupus erythematosus (SLE), while terminal component deficiency is well known to predispose to recurrent neisserial infection. In contrast, only recently have patients been reported with rheumatic disease and hereditary deficiency of a terminal component. T...

Beta-2A globulin and the 7S and 19S gamma globulins are the major proteins of the serum derived from the immune system. Collectively they have been termed the immunoglobulins. The 19S gamma globulin, also known as the gamma-1 macroglobulin, the 82 macroglobulin or 82M, has been the subject of intensive study in recent years. The rheumatoid factor as well as a variety of anti-bodies fall into th...

the central immunological disturbance in systemic lupus erythematosus (sle) is autoantibody production. some of these antibodies affecting components of the cell nucleus are the major characteristics of sle. the present study was aimed to assess importance of anticardiolipin (acl) antibody and its association with clinical state in sle patients. a cross sectional study was performed on 100 pati...

Evans, D. I. K., and Holzel, A. (1970). Archives of Disease in Childhood, 45, 527. Immune deficiency state in a girl with eczema and low serum IgM. Possible female variant of Wiskott-Aldrich syndrome. This report concerns an immune deficiency disorder in a girl with eczema. She has had recurrent infections including three severe attacks of herpes simplex and five attacks of pneumococcal meningi...

Evans, D. I. K., and Holzel, A. (1970). Archives of Disease in Childhood, 45, 527. Immune deficiency state in a girl with eczema and low serum IgM. Possible female variant of Wiskott-Aldrich syndrome. This report concerns an immune deficiency disorder in a girl with eczema. She has had recurrent infections including three severe attacks of herpes simplex and five attacks of pneumococcal meningi...

the hallmarks of leukocyte adhesion deficiency (lad) are defects in the leukocyte adhesion process, marked leukocytosis and recurrent infections. these molecular and clinical manifestations result from an impaired step in the inflammatory process, namely, the emigration of leukocytes from the blood vessels to sites of infection, which requires adhesion of leukocytes to the endothelium. over las...

A 24 year old man presented with an unusual primary combined immune deficiency syndrome characterised by a profound lymphopenia of CD4 cells, selective serum IgG2 subclass deficiency, poor polysaccharide antibody responses, disseminated warts, recurrent sinopulmonary infection and bronchiectasis. The developed progressive multifocal leucoencephalopathy (PML) in association with sclerosing chola...

background: hyper ige syndrome (hies) is a rare primary immune deficiency, described as job`s syndrome characterized by increased serum levels of ige, eczema, recurrent cutaneous and pulmonary infections. in this paper, we presented a case of hyper ige syndrome.case presentation: a 16-year-old iranian boy presented with a one year history of skin lesions in knees and elbows was diagnosed of pso...